Details for: HMBS

Gene ID: 3145

Symbol: HMBS

Ensembl ID: ENSG00000256269

Description: hydroxymethylbilane synthase

Associated with

Cells (max top 100)

(Cell Significance Index and respective Thresholds are uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: basophilic erythroblast (CL0000549)
    Fold Change: 64.7334
    Cell Significance Index: 29.4800
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 40.5293
    Cell Significance Index: -10.2800
  • Cell Name: mucosal type mast cell (CL0000485)
    Fold Change: 28.9472
    Cell Significance Index: -11.7600
  • Cell Name: ileal goblet cell (CL1000326)
    Fold Change: 20.9829
    Cell Significance Index: -14.0800
  • Cell Name: ciliated cell of the bronchus (CL0002332)
    Fold Change: 12.3698
    Cell Significance Index: -11.8100
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 4.6475
    Cell Significance Index: -12.4500
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 3.4592
    Cell Significance Index: -13.6500
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 1.6459
    Cell Significance Index: 113.8200
  • Cell Name: epidermal Langerhans cell (CL0002457)
    Fold Change: 1.3296
    Cell Significance Index: -2.9100
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 1.0541
    Cell Significance Index: 114.6600
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 1.0451
    Cell Significance Index: 943.6800
  • Cell Name: cortical cell of adrenal gland (CL0002097)
    Fold Change: 0.9425
    Cell Significance Index: 25.2600
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 0.8240
    Cell Significance Index: 134.0200
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: 0.7312
    Cell Significance Index: 85.2100
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: 0.6824
    Cell Significance Index: 30.9300
  • Cell Name: primitive red blood cell (CL0002355)
    Fold Change: 0.6303
    Cell Significance Index: 7.1400
  • Cell Name: skeletal muscle myoblast (CL0000515)
    Fold Change: 0.5657
    Cell Significance Index: 6.1500
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 0.4818
    Cell Significance Index: 91.6800
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: 0.4794
    Cell Significance Index: 30.2200
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: 0.4720
    Cell Significance Index: 6.4400
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.3935
    Cell Significance Index: 20.4400
  • Cell Name: paneth cell of colon (CL0009009)
    Fold Change: 0.3844
    Cell Significance Index: 5.7600
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.3773
    Cell Significance Index: 10.8700
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.3401
    Cell Significance Index: 185.7200
  • Cell Name: sebum secreting cell (CL0000317)
    Fold Change: 0.3159
    Cell Significance Index: 22.3400
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: 0.3009
    Cell Significance Index: 8.4100
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: 0.3005
    Cell Significance Index: 6.5100
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 0.2964
    Cell Significance Index: 13.8200
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: 0.2797
    Cell Significance Index: 32.9900
  • Cell Name: retinal rod cell (CL0000604)
    Fold Change: 0.2663
    Cell Significance Index: 3.1800
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: 0.2580
    Cell Significance Index: 6.8900
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: 0.2258
    Cell Significance Index: 99.8200
  • Cell Name: fibroblast of dermis (CL0002551)
    Fold Change: 0.2116
    Cell Significance Index: 4.4300
  • Cell Name: transit amplifying cell of small intestine (CL0009012)
    Fold Change: 0.2068
    Cell Significance Index: 4.2900
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: 0.1983
    Cell Significance Index: 6.3500
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.1871
    Cell Significance Index: 37.1300
  • Cell Name: mesonephric nephron tubule epithelial cell (CL1000022)
    Fold Change: 0.1714
    Cell Significance Index: 5.9600
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: 0.1667
    Cell Significance Index: 30.0500
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: 0.1529
    Cell Significance Index: 18.8100
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: 0.1474
    Cell Significance Index: 3.1400
  • Cell Name: CD4-positive, alpha-beta memory T cell, CD45RO-positive (CL0001204)
    Fold Change: 0.1103
    Cell Significance Index: 3.2400
  • Cell Name: epithelial cell of uterus (CL0002149)
    Fold Change: 0.1099
    Cell Significance Index: 1.5300
  • Cell Name: lactocyte (CL0002325)
    Fold Change: 0.1068
    Cell Significance Index: 13.8000
  • Cell Name: basal cell of prostate epithelium (CL0002341)
    Fold Change: 0.0871
    Cell Significance Index: 2.3700
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.0828
    Cell Significance Index: 5.3400
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 0.0818
    Cell Significance Index: 8.0900
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 0.0702
    Cell Significance Index: 14.0900
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: 0.0613
    Cell Significance Index: 0.6400
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: 0.0611
    Cell Significance Index: 42.2500
  • Cell Name: peg cell (CL4033014)
    Fold Change: 0.0519
    Cell Significance Index: 1.2000
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: 0.0505
    Cell Significance Index: 3.7600
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 0.0330
    Cell Significance Index: 4.5300
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: 0.0311
    Cell Significance Index: 1.4600
  • Cell Name: mesenchymal cell (CL0008019)
    Fold Change: 0.0299
    Cell Significance Index: 0.5000
  • Cell Name: odontoblast (CL0000060)
    Fold Change: 0.0297
    Cell Significance Index: 3.8100
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: 0.0242
    Cell Significance Index: 18.3400
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: 0.0213
    Cell Significance Index: 7.6300
  • Cell Name: kidney cell (CL1000497)
    Fold Change: 0.0200
    Cell Significance Index: 0.1600
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: 0.0028
    Cell Significance Index: 0.4700
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: -0.0041
    Cell Significance Index: -7.6900
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: -0.0055
    Cell Significance Index: -4.0200
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: -0.0069
    Cell Significance Index: -12.6800
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: -0.0078
    Cell Significance Index: -11.9600
  • Cell Name: secondary lens fiber (CL0002225)
    Fold Change: -0.0109
    Cell Significance Index: -14.8400
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: -0.0146
    Cell Significance Index: -9.2800
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0162
    Cell Significance Index: -11.9700
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: -0.0203
    Cell Significance Index: -2.0700
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.0232
    Cell Significance Index: -10.5500
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0232
    Cell Significance Index: -13.1000
  • Cell Name: hepatoblast (CL0005026)
    Fold Change: -0.0232
    Cell Significance Index: -0.3900
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: -0.0273
    Cell Significance Index: -17.0400
  • Cell Name: pro-T cell (CL0000827)
    Fold Change: -0.0442
    Cell Significance Index: -1.1300
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: -0.0448
    Cell Significance Index: -3.4400
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.0480
    Cell Significance Index: -13.8000
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: -0.0512
    Cell Significance Index: -2.6900
  • Cell Name: umbrella cell of urothelium (CL4030056)
    Fold Change: -0.0570
    Cell Significance Index: -0.5300
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.0663
    Cell Significance Index: -7.6000
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: -0.0706
    Cell Significance Index: -2.4800
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.0722
    Cell Significance Index: -15.2100
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.0755
    Cell Significance Index: -10.9800
  • Cell Name: microcirculation associated smooth muscle cell (CL0008035)
    Fold Change: -0.0940
    Cell Significance Index: -0.7900
  • Cell Name: endothelial cell of venule (CL1000414)
    Fold Change: -0.1100
    Cell Significance Index: -1.2500
  • Cell Name: granulosa cell (CL0000501)
    Fold Change: -0.1175
    Cell Significance Index: -3.0900
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.1253
    Cell Significance Index: -7.0300
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.1277
    Cell Significance Index: -13.3000
  • Cell Name: lung endothelial cell (CL1001567)
    Fold Change: -0.1350
    Cell Significance Index: -7.0300
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: -0.1400
    Cell Significance Index: -11.0900
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: -0.1432
    Cell Significance Index: -3.8300
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.1519
    Cell Significance Index: -9.3100
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: -0.1528
    Cell Significance Index: -9.3900
  • Cell Name: hippocampal granule cell (CL0001033)
    Fold Change: -0.1728
    Cell Significance Index: -11.6200
  • Cell Name: reticulocyte (CL0000558)
    Fold Change: -0.1806
    Cell Significance Index: -0.9500
  • Cell Name: proerythroblast (CL0000547)
    Fold Change: -0.1821
    Cell Significance Index: -2.6100
  • Cell Name: kidney epithelial cell (CL0002518)
    Fold Change: -0.2329
    Cell Significance Index: -6.8600
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: -0.2468
    Cell Significance Index: -6.1700
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.2516
    Cell Significance Index: -11.1300
  • Cell Name: skeletal muscle fiber (CL0008002)
    Fold Change: -0.2521
    Cell Significance Index: -6.4800
  • Cell Name: cerebellar granule cell (CL0001031)
    Fold Change: -0.2638
    Cell Significance Index: -4.5200
  • Cell Name: germ cell (CL0000586)
    Fold Change: -0.2649
    Cell Significance Index: -2.0000
  • Cell Name: L5 extratelencephalic projecting glutamatergic cortical neuron (CL4023041)
    Fold Change: -0.2937
    Cell Significance Index: -10.2900

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this specific cell.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Cell ID: Standard Cell Ontology term used for mapping and comparing cells across experiments. Ensures consistency in analyzing cellular functions across tissues.
Fold Change: Represents the ratio of the current Cell Significance Index to the Cell Significance Index Threshold, indicating how much the gene expression has changed compared to a baseline.
Cell Significance Index: Reflects how strongly a gene is expressed in this cell type. Calculated using techniques like effect size estimation and bootstrapping for reliability.

Other Information

**Key Characteristics:** 1. **Heme Biosynthesis:** HMBS is a key enzyme in the heme biosynthetic pathway, catalyzing the formation of hydroxymethylbilane from ALA and succinyl-CoA. 2. **Cellular Expression:** HMBS is significantly expressed in erythroid progenitor cells, proerythroblasts, placental villous trophoblasts, and other cell types involved in hematopoiesis and development. 3. **Protein Binding:** HMBS interacts with various proteins, including those involved in heme biosynthesis, protein folding, and metabolism of porphyrins. 4. **Metabolic Pathways:** HMBS is involved in the metabolism of porphyrins, a class of compounds essential for heme synthesis and other cellular processes. **Pathways and Functions:** 1. **Heme Biosynthesis:** HMBS catalyzes the formation of hydroxymethylbilane, a precursor to heme, in the heme biosynthetic pathway. 2. **Protoporphyrinogen IX Biosynthesis:** HMBS is also involved in the biosynthesis of protoporphyrinogen IX, a crucial intermediate in the heme biosynthetic pathway. 3. **Protein Binding:** HMBS interacts with proteins involved in heme biosynthesis, protein folding, and metabolism of porphyrins, highlighting its role in regulating these processes. 4. **Metabolism of Porphyrins:** HMBS is involved in the metabolism of porphyrins, a class of compounds essential for heme synthesis and other cellular processes. **Clinical Significance:** Dysregulation of HMBS has been implicated in various hematological disorders, including erythropoietic protoporphyria (EPP) and porphyria cutanea tarda (PCT). Mutations in the HMBS gene have been associated with these conditions, highlighting the importance of this enzyme in heme biosynthesis and cellular metabolism. Additionally, HMBS has been shown to play a role in the development of certain types of cancer, including leukemia and lymphoma. Further research is needed to fully elucidate the clinical significance of HMBS and its role in human disease. In conclusion, HMBS is a critical enzyme in the heme biosynthetic pathway, with a wide range of cellular expressions and interactions. Its dysregulation has been implicated in various hematological disorders and cancer, highlighting the importance of this enzyme in human health and disease. Further research is needed to fully understand the role of HMBS in heme biosynthesis and its implications for human disease.

Genular Protein ID: 3121600223

Symbol: HEM3_HUMAN

Name: Hydroxymethylbilane synthase

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 2875434

Title: Molecular cloning and complete primary sequence of human erythrocyte porphobilinogen deaminase.

PubMed ID: 2875434

DOI: 10.1093/nar/14.15.5955

PubMed ID: 3816774

Title: Tissue-specific expression of porphobilinogen deaminase. Two isoenzymes from a single gene.

PubMed ID: 3816774

DOI: 10.1111/j.1432-1033.1987.tb10548.x

PubMed ID: 7916736

Title: Hydroxymethylbilane synthase: complete genomic sequence and amplifiable polymorphisms in the human gene.

PubMed ID: 7916736

DOI: 10.1006/geno.1993.1005

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 16554811

Title: Human chromosome 11 DNA sequence and analysis including novel gene identification.

PubMed ID: 16554811

DOI: 10.1038/nature04632

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 3422427

Title: Alternative transcription and splicing of the human porphobilinogen deaminase gene result either in tissue-specific or in housekeeping expression.

PubMed ID: 3422427

DOI: 10.1073/pnas.85.1.6

PubMed ID: 2609111

Title: Porphobilinogen deaminase in human erythrocytes: purification of two forms with apparent molecular weights of 40 kDa and 42 kDa.

PubMed ID: 2609111

DOI: 10.3109/00365518909091544

PubMed ID: 18004775

Title: Human uroporphyrinogen III synthase: NMR-based mapping of the active site.

PubMed ID: 18004775

DOI: 10.1002/prot.21755

PubMed ID: 19413330

Title: Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.

PubMed ID: 19413330

DOI: 10.1021/ac9004309

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 22223895

Title: Comparative large-scale characterisation of plant vs. mammal proteins reveals similar and idiosyncratic N-alpha acetylation features.

PubMed ID: 22223895

DOI: 10.1074/mcp.m111.015131

PubMed ID: 22814378

Title: N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB.

PubMed ID: 22814378

DOI: 10.1073/pnas.1210303109

PubMed ID: 23186163

Title: Toward a comprehensive characterization of a human cancer cell phosphoproteome.

PubMed ID: 23186163

DOI: 10.1021/pr300630k

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 19207107

Title: Structure of human porphobilinogen deaminase at 2.8 A: the molecular basis of acute intermittent porphyria.

PubMed ID: 19207107

DOI: 10.1042/bj20082077

PubMed ID: 18936296

Title: Structural insight into acute intermittent porphyria.

PubMed ID: 18936296

DOI: 10.1096/fj.08-115469

PubMed ID: 2243128

Title: Two different point G to A mutations in exon 10 of the porphobilinogen deaminase gene are responsible for acute intermittent porphyria.

PubMed ID: 2243128

DOI: 10.1172/jci114869

PubMed ID: 1714233

Title: Molecular heterogeneity of acute intermittent porphyria: identification of four additional mutations resulting in the CRIM-negative subtype of the disease.

PubMed ID: 1714233

PubMed ID: 1496994

Title: High frequency of mutations in exon 10 of the porphobilinogen deaminase gene in patients with a CRIM-positive subtype of acute intermittent porphyria.

PubMed ID: 1496994

PubMed ID: 1577472

Title: Homozygous acute intermittent porphyria: compound heterozygosity for adjacent base transitions in the same codon of the porphobilinogen deaminase gene.

PubMed ID: 1577472

DOI: 10.1007/bf00207051

PubMed ID: 1427766

Title: Detection of seven point mutations in the porphobilinogen deaminase gene in patients with acute intermittent porphyria, by direct sequencing of in vitro amplified cDNA.

PubMed ID: 1427766

DOI: 10.1007/bf00210738

PubMed ID: 1301948

Title: CRIM-positive mutations of acute intermittent porphyria in Finland.

PubMed ID: 1301948

DOI: 10.1002/humu.1380010508

PubMed ID: 8262523

Title: Detection of a high mutation frequency in exon 12 of the porphobilinogen deaminase gene in patients with acute intermittent porphyria.

PubMed ID: 8262523

DOI: 10.1007/bf00420949

PubMed ID: 8401516

Title: Acute intermittent porphyria caused by an arginine to histidine substitution (R26H) in the cofactor-binding cleft of porphobilinogen deaminase.

PubMed ID: 8401516

DOI: 10.1093/hmg/2.8.1315

PubMed ID: 8268934

Title: Two novel mutations of the porphobilinogen deaminase gene in acute intermittent porphyria.

PubMed ID: 8268934

DOI: 10.1093/hmg/2.10.1735

PubMed ID: 8270254

Title: Detection of eleven mutations causing acute intermittent porphyria using denaturing gradient gel electrophoresis.

PubMed ID: 8270254

DOI: 10.1007/bf00218912

PubMed ID: 8270256

Title: Two new mutations in the porphobilinogen deaminase gene and a screening method using PCR amplification of specific alleles.

PubMed ID: 8270256

DOI: 10.1007/bf00218914

PubMed ID: 8081367

Title: Identification of five novel mutations in the porphobilinogen deaminase gene.

PubMed ID: 8081367

DOI: 10.1093/hmg/3.5.809

PubMed ID: 7866402

Title: Molecular basis of acute intermittent porphyria: mutations and polymorphisms in the human hydroxymethylbilane synthase gene.

PubMed ID: 7866402

DOI: 10.1002/humu.1380040403

PubMed ID: 7962538

Title: Acute intermittent porphyria: identification and expression of exonic mutations in the hydroxymethylbilane synthase gene. An initiation codon missense mutation in the housekeeping transcript causes 'variant acute intermittent porphyria' with normal expression of the erythroid-specific enzyme.

PubMed ID: 7962538

DOI: 10.1172/jci117543

PubMed ID: 7757070

Title: Acute intermittent porphyria in Finland: 19 mutations in the porphobilinogen deaminase gene.

PubMed ID: 7757070

DOI: 10.1093/hmg/4.2.215

PubMed ID: 8825929

Title: Four mutations in the porphobilinogen deaminase gene in patients with acute intermittent porphyria.

PubMed ID: 8825929

DOI: 10.1136/jmg.32.12.979

PubMed ID: 9199558

Title: Molecular epidemiology and diagnosis of PBG deaminase gene defects in acute intermittent porphyria.

PubMed ID: 9199558

DOI: 10.1086/515455

PubMed ID: 9225970

Title: Genetic investigation of the porphobilinogen deaminase gene in Swedish acute intermittent porphyria families.

PubMed ID: 9225970

DOI: 10.1007/s004390050466

PubMed ID: 9654202

Title: Three splicing defects, an insertion, and two missense mutations responsible for acute intermittent porphyria.

PubMed ID: 9654202

DOI: 10.1007/s004390050737

PubMed ID: 9463797

Title: Identification of two novel mutations in the hydroxymethylbilane synthase gene in three patients from two unrelated families with acute intermittent porphyria.

PubMed ID: 9463797

DOI: 10.1159/000022777

PubMed ID: 10494093

Title: Identification and characterization of hydroxymethylbilane synthase mutations causing acute intermittent porphyria: evidence for an ancestral founder of the common G111R mutation.

PubMed ID: 10494093

DOI: 10.1002/(sici)1096-8628(19991008)86:4<366::aid-ajmg11>3.3.co;2-r

PubMed ID: 10453740

Title: Comparison of complementary and genomic DNA sequencing for the detection of mutations in the HMBS gene in British patients with acute intermittent porphyria: identification of 25 novel mutations.

PubMed ID: 10453740

DOI: 10.1007/s004390050995

PubMed ID: 10502788

Title: Acute intermittent porphyria: characterization of two novel mutations in the porphobilinogen deaminase gene, one amino acid deletion (453-455delAGC) and one splicing acceptor site mutation (IVS8-1G>T).

PubMed ID: 10502788

DOI: 10.1002/(sici)1098-1004(199910)14:4<355::aid-humu19>3.0.co;2-t

PubMed ID: 10657149

Title: New mutations of the hydroxymethylbilane synthase gene in German patients with acute intermittent porphyria.

PubMed ID: 10657149

DOI: 10.1006/mcpr.1999.0276

PubMed ID: 10602775

Title: Identification and expression of mutations in the hydroxymethylbilane synthase gene causing acute intermittent porphyria (AIP).

PubMed ID: 10602775

DOI: 10.1007/bf03401985

PubMed ID: 11399210

Title: Acute intermittent porphyria: novel missense mutations in the human hydroxymethylbilane synthase gene.

PubMed ID: 11399210

DOI: 10.1097/00125817-200009000-00004

PubMed ID: 11030413

Title: Porphobilinogen deaminase gene in African and Afro-Caribbean ethnic groups: mutations causing acute intermittent porphyria and specific intragenic polymorphisms.

PubMed ID: 11030413

DOI: 10.1007/s004390000323

PubMed ID: 10782018

Title: Identification of a prevalent nonsense mutation (W283X) and two novel mutations in the porphobilinogen deaminase gene of Swiss patients with acute intermittent porphyria.

PubMed ID: 10782018

DOI: 10.1159/000022924

PubMed ID: 11013452

Title: Identification and characterization of two novel mutations that produce acute intermittent porphyria: a 3-base deletion (841-843delGGA) and a missense mutation (T35M).

PubMed ID: 11013452

DOI: 10.1002/1098-1004(200010)16:4<373::aid-humu14>3.0.co;2-a

PubMed ID: 12406973

Title: Molecular and biochemical studies of acute intermittent porphyria in 196 patients and their families.

PubMed ID: 12406973

PubMed ID: 12372055

Title: Acute intermittent porphyria in Sweden. Molecular, functional and clinical consequences of some new mutations found in the porphobilinogen deaminase gene.

PubMed ID: 12372055

DOI: 10.1034/j.1399-0004.2002.620406.x

PubMed ID: 11857754

Title: Molecular study of the hydroxymethylbilane synthase gene (HMBS) among Polish patients with acute intermittent porphyria.

PubMed ID: 11857754

DOI: 10.1002/humu.9020

PubMed ID: 12773194

Title: Human porphobilinogen deaminase mutations in the investigation of the mechanism of dipyrromethane cofactor assembly and tetrapyrrole formation.

PubMed ID: 12773194

DOI: 10.1042/bst0310731

PubMed ID: 14757946

Title: Haplotype analysis of Norwegian and Swedish patients with acute intermittent porphyria (AIP): Extreme haplotype heterogeneity for the mutation R116W.

PubMed ID: 14757946

DOI: 10.1155/2003/384971

PubMed ID: 14669009

Title: Modulation of penetrance by the wild-type allele in dominantly inherited erythropoietic protoporphyria and acute hepatic porphyrias.

PubMed ID: 14669009

DOI: 10.1007/s00439-003-1059-5

PubMed ID: 14970743

Title: Homozygous acute intermittent porphyria in a 7-year-old boy with massive excretions of porphyrins and porphyrin precursors.

PubMed ID: 14970743

DOI: 10.1023/b:boli.0000016613.75677.05

PubMed ID: 15669678

Title: Mutation hotspots in the human porphobilinogen deaminase gene: recurrent mutations G111R and R173Q occurring at CpG motifs.

PubMed ID: 15669678

DOI: 10.1023/b:boli.0000042936.20691.ad

PubMed ID: 16211556

Title: Nine mutations including three novel mutations among Russian patients with acute intermittent porphyria.

PubMed ID: 16211556

DOI: 10.1002/humu.9381

PubMed ID: 18406650

Title: Characterization of two missense variants in the hydroxymethylbilane synthase gene in the Israeli population, which differ in their associations with acute intermittent porphyria.

PubMed ID: 18406650

DOI: 10.1016/j.ymgme.2008.03.001

PubMed ID: 19138865

Title: Correlation between biochemical findings, structural and enzymatic abnormalities in mutated HMBS identified in six Israeli families with acute intermittent porphyria.

PubMed ID: 19138865

DOI: 10.1016/j.bcmd.2008.11.001

PubMed ID: 19292878

Title: Acute intermittent porphyria--impact of mutations found in the hydroxymethylbilane synthase gene on biochemical and enzymatic protein properties.

PubMed ID: 19292878

DOI: 10.1111/j.1742-4658.2009.06946.x

PubMed ID: 23815679

Title: Conformational stability and activity analysis of two hydroxymethylbilane synthase mutants, K132N and V215E, with different phenotypic association with acute intermittent porphyria.

PubMed ID: 23815679

DOI: 10.1042/bsr20130045

PubMed ID: 25703257

Title: Hydroxymethylbilane synthase gene mutations and polymorphisms in Brazilian families with acute intermittent porphyria.

PubMed ID: 25703257

DOI: 10.1111/ahg.12102

PubMed ID: 25870942

Title: A novel mutation in the porphobilinogen deaminase gene in an extended Chinese family with acute intermittent porphyria.

PubMed ID: 25870942

DOI: 10.1016/j.gene.2015.04.027

PubMed ID: 27558376

Title: Acute intermittent porphyria-related leukoencephalopathy.

PubMed ID: 27558376

DOI: 10.1212/wnl.0000000000003129

PubMed ID: 29360981

Title: From a dominant to an oligogenic model of inheritance with environmental modifiers in acute intermittent porphyria.

PubMed ID: 29360981

DOI: 10.1093/hmg/ddy030

PubMed ID: 34089223

Title: Expanding the clinical and radiological phenotypes of leukoencephalopathy due to biallelic HMBS mutations.

PubMed ID: 34089223

DOI: 10.1002/ajmg.a.62377

Sequence Information:

  • Length: 361
  • Mass: 39330
  • Checksum: 8F2F6F4150F1AD7E
  • Sequence:
  • MSGNGNAAAT AEENSPKMRV IRVGTRKSQL ARIQTDSVVA TLKASYPGLQ FEIIAMSTTG 
    DKILDTALSK IGEKSLFTKE LEHALEKNEV DLVVHSLKDL PTVLPPGFTI GAICKRENPH 
    DAVVFHPKFV GKTLETLPEK SVVGTSSLRR AAQLQRKFPH LEFRSIRGNL NTRLRKLDEQ 
    QEFSAIILAT AGLQRMGWHN RVGQILHPEE CMYAVGQGAL GVEVRAKDQD ILDLVGVLHD 
    PETLLRCIAE RAFLRHLEGG CSVPVAVHTA MKDGQLYLTG GVWSLDGSDS IQETMQATIH 
    VPAQHEDGPE DDPQLVGITA RNIPRGPQLA AQNLGISLAN LLLSKGAKNI LDVARQLNDA 
    H

Genular Protein ID: 1630448330

Symbol: A0A8I5KXV4_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 11237011

Title: Initial sequencing and analysis of the human genome.

PubMed ID: 11237011

DOI: 10.1038/35057062

PubMed ID: 15496913

Title: Finishing the euchromatic sequence of the human genome.

PubMed ID: 15496913

DOI: 10.1038/nature03001

PubMed ID: 16554811

Title: Human chromosome 11 DNA sequence and analysis including novel gene identification.

PubMed ID: 16554811

DOI: 10.1038/nature04632

Sequence Information:

  • Length: 306
  • Mass: 33499
  • Checksum: 3CF234BA58375C53
  • Sequence:
  • MSTTGDKILD TALSKIGEKS LFTKELEHAL EKNEVDLVVH SLKDLPTVLP PGFTIGAICK 
    RENPHDAVVF HPKFVGKTLE TLPEKSVVGT SSLRRAAQLQ RKFPHLEFRS IRGNLNTRLR 
    KLDEQQEFSA IILATAGLQR MGWHNRVGQI LHPEECMYAV GQGALGVEVR AKDQDILDLV 
    GVLHDPETLL RCIAERAFLR HLEGGCSVPV AVHTAMKDGQ LYLTGGVWSL DGSDSIQETM 
    QATIHVPAQH EDGPEDDPQL VGITARNIPR GPQLAAQNLG ISLANLLLSK GAKNILDVAR 
    QLNDAH

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.