Details for: OTC

Gene ID: 5009

Symbol: OTC

Ensembl ID: ENSG00000036473

Description: ornithine transcarbamylase

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: precursor cell (CL0011115)
    Fold Change: 4.9683
    Cell Significance Index: 37.6800
  • Cell Name: hepatoblast (CL0005026)
    Fold Change: 3.9205
    Cell Significance Index: 65.9400
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 3.3111
    Cell Significance Index: -8.8700
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 3.2349
    Cell Significance Index: 615.6200
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 2.8518
    Cell Significance Index: 82.1700
  • Cell Name: gut absorptive cell (CL0000677)
    Fold Change: 2.0678
    Cell Significance Index: 124.1400
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 1.7884
    Cell Significance Index: 176.9200
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: 1.6963
    Cell Significance Index: 36.7500
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 1.5447
    Cell Significance Index: 168.0200
  • Cell Name: tuft cell of small intestine (CL0009080)
    Fold Change: 1.4770
    Cell Significance Index: 14.9000
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: 1.4518
    Cell Significance Index: 30.9200
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: 1.2570
    Cell Significance Index: 44.1700
  • Cell Name: CD14-low, CD16-positive monocyte (CL0002396)
    Fold Change: 1.1981
    Cell Significance Index: 29.0200
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 1.1723
    Cell Significance Index: 1058.4900
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 1.0308
    Cell Significance Index: 167.6600
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 0.8424
    Cell Significance Index: 58.2600
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: 0.4837
    Cell Significance Index: 12.0900
  • Cell Name: CD14-positive monocyte (CL0001054)
    Fold Change: 0.2519
    Cell Significance Index: 4.9300
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: 0.2474
    Cell Significance Index: 171.1400
  • Cell Name: plasmablast (CL0000980)
    Fold Change: 0.1390
    Cell Significance Index: 1.2900
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: 0.1092
    Cell Significance Index: 1.1300
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: 0.1046
    Cell Significance Index: 3.3500
  • Cell Name: colonocyte (CL1000347)
    Fold Change: 0.0291
    Cell Significance Index: 0.1800
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 0.0067
    Cell Significance Index: 1.3400
  • Cell Name: periportal region hepatocyte (CL0019026)
    Fold Change: 0.0027
    Cell Significance Index: 0.0400
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: 0.0026
    Cell Significance Index: 0.1200
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: -0.0020
    Cell Significance Index: -0.3500
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: -0.0065
    Cell Significance Index: -2.3200
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0075
    Cell Significance Index: -5.5300
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: -0.0095
    Cell Significance Index: -7.1600
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0098
    Cell Significance Index: -5.5200
  • Cell Name: GABAergic neuron (CL0000617)
    Fold Change: -0.0111
    Cell Significance Index: -0.1400
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: -0.0118
    Cell Significance Index: -8.6700
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: -0.0240
    Cell Significance Index: -3.3000
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.0246
    Cell Significance Index: -1.5100
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.0280
    Cell Significance Index: -3.2100
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.0280
    Cell Significance Index: -5.8900
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.0293
    Cell Significance Index: -8.4400
  • Cell Name: BEST4+ enteroycte (CL4030026)
    Fold Change: -0.0405
    Cell Significance Index: -0.6100
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: -0.0408
    Cell Significance Index: -4.7600
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: -0.0448
    Cell Significance Index: -8.0700
  • Cell Name: centrilobular region hepatocyte (CL0019029)
    Fold Change: -0.0558
    Cell Significance Index: -0.9400
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: -0.0568
    Cell Significance Index: -6.9800
  • Cell Name: fibro/adipogenic progenitor cell (CL0009099)
    Fold Change: -0.0656
    Cell Significance Index: -3.3100
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: -0.0674
    Cell Significance Index: -5.1700
  • Cell Name: lactocyte (CL0002325)
    Fold Change: -0.0680
    Cell Significance Index: -8.7800
  • Cell Name: fibroblast of dermis (CL0002551)
    Fold Change: -0.0693
    Cell Significance Index: -1.4500
  • Cell Name: neuron associated cell (CL0000095)
    Fold Change: -0.0772
    Cell Significance Index: -3.1600
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: -0.0778
    Cell Significance Index: -4.7900
  • Cell Name: medial ganglionic eminence derived interneuron (CL4023063)
    Fold Change: -0.0943
    Cell Significance Index: -1.3500
  • Cell Name: stem cell (CL0000034)
    Fold Change: -0.1032
    Cell Significance Index: -0.7500
  • Cell Name: nasal mucosa goblet cell (CL0002480)
    Fold Change: -0.1113
    Cell Significance Index: -1.1400
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: -0.1123
    Cell Significance Index: -6.3000
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: -0.1175
    Cell Significance Index: -8.7600
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: -0.1209
    Cell Significance Index: -6.3500
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.1223
    Cell Significance Index: -5.4100
  • Cell Name: L6b glutamatergic cortical neuron (CL4023038)
    Fold Change: -0.1228
    Cell Significance Index: -4.0200
  • Cell Name: L5 extratelencephalic projecting glutamatergic cortical neuron (CL4023041)
    Fold Change: -0.1240
    Cell Significance Index: -4.3500
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: -0.1265
    Cell Significance Index: -5.9000
  • Cell Name: corticothalamic-projecting glutamatergic cortical neuron (CL4023013)
    Fold Change: -0.1272
    Cell Significance Index: -4.0500
  • Cell Name: skeletal muscle myoblast (CL0000515)
    Fold Change: -0.1315
    Cell Significance Index: -1.4300
  • Cell Name: medium spiny neuron (CL1001474)
    Fold Change: -0.1378
    Cell Significance Index: -1.8600
  • Cell Name: ionocyte (CL0005006)
    Fold Change: -0.1396
    Cell Significance Index: -1.4600
  • Cell Name: enterocyte (CL0000584)
    Fold Change: -0.1402
    Cell Significance Index: -0.8700
  • Cell Name: L2/3 intratelencephalic projecting glutamatergic neuron (CL4030059)
    Fold Change: -0.1448
    Cell Significance Index: -1.9300
  • Cell Name: myoblast (CL0000056)
    Fold Change: -0.1455
    Cell Significance Index: -1.4300
  • Cell Name: direct pathway medium spiny neuron (CL4023026)
    Fold Change: -0.1468
    Cell Significance Index: -5.5600
  • Cell Name: osteoblast (CL0000062)
    Fold Change: -0.1474
    Cell Significance Index: -1.4300
  • Cell Name: muscle cell (CL0000187)
    Fold Change: -0.1495
    Cell Significance Index: -1.4300
  • Cell Name: L4 intratelencephalic projecting glutamatergic neuron (CL4030063)
    Fold Change: -0.1505
    Cell Significance Index: -1.6500
  • Cell Name: decidual cell (CL2000002)
    Fold Change: -0.1527
    Cell Significance Index: -2.4500
  • Cell Name: immature NK T cell (CL0000914)
    Fold Change: -0.1617
    Cell Significance Index: -2.0700
  • Cell Name: epithelial cell of esophagus (CL0002252)
    Fold Change: -0.1657
    Cell Significance Index: -1.1000
  • Cell Name: cortical cell of adrenal gland (CL0002097)
    Fold Change: -0.1698
    Cell Significance Index: -4.5500
  • Cell Name: pulmonary capillary endothelial cell (CL4028001)
    Fold Change: -0.1716
    Cell Significance Index: -2.3500
  • Cell Name: pvalb GABAergic cortical interneuron (CL4023018)
    Fold Change: -0.1720
    Cell Significance Index: -3.6500
  • Cell Name: near-projecting glutamatergic cortical neuron (CL4023012)
    Fold Change: -0.1756
    Cell Significance Index: -4.3800
  • Cell Name: endothelial cell of uterus (CL0009095)
    Fold Change: -0.1781
    Cell Significance Index: -1.1200
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: -0.1790
    Cell Significance Index: -8.4200
  • Cell Name: transit amplifying cell of small intestine (CL0009012)
    Fold Change: -0.1798
    Cell Significance Index: -3.7300
  • Cell Name: progenitor cell (CL0011026)
    Fold Change: -0.1845
    Cell Significance Index: -1.9600
  • Cell Name: natural T-regulatory cell (CL0000903)
    Fold Change: -0.1909
    Cell Significance Index: -1.8700
  • Cell Name: stratified epithelial cell (CL0000079)
    Fold Change: -0.1931
    Cell Significance Index: -7.0900
  • Cell Name: cerebral cortex neuron (CL0010012)
    Fold Change: -0.1976
    Cell Significance Index: -1.8800
  • Cell Name: paneth cell of colon (CL0009009)
    Fold Change: -0.1995
    Cell Significance Index: -2.9900
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: -0.2007
    Cell Significance Index: -5.3600
  • Cell Name: lamp5 GABAergic cortical interneuron (CL4023011)
    Fold Change: -0.2016
    Cell Significance Index: -4.3600
  • Cell Name: VIP GABAergic cortical interneuron (CL4023016)
    Fold Change: -0.2030
    Cell Significance Index: -4.0800
  • Cell Name: cerebral cortex endothelial cell (CL1001602)
    Fold Change: -0.2062
    Cell Significance Index: -4.2000
  • Cell Name: cerebral cortex GABAergic interneuron (CL0010011)
    Fold Change: -0.2095
    Cell Significance Index: -2.1100
  • Cell Name: urothelial cell (CL0000731)
    Fold Change: -0.2108
    Cell Significance Index: -1.4700
  • Cell Name: cone retinal bipolar cell (CL0000752)
    Fold Change: -0.2147
    Cell Significance Index: -1.6600
  • Cell Name: retinal blood vessel endothelial cell (CL0002585)
    Fold Change: -0.2206
    Cell Significance Index: -2.8500
  • Cell Name: keratinocyte (CL0000312)
    Fold Change: -0.2258
    Cell Significance Index: -5.6400
  • Cell Name: sst GABAergic cortical interneuron (CL4023017)
    Fold Change: -0.2261
    Cell Significance Index: -4.4700
  • Cell Name: type G enteroendocrine cell (CL0000508)
    Fold Change: -0.2285
    Cell Significance Index: -2.4200
  • Cell Name: thymocyte (CL0000893)
    Fold Change: -0.2291
    Cell Significance Index: -2.9000
  • Cell Name: peptic cell (CL0000155)
    Fold Change: -0.2318
    Cell Significance Index: -2.0600
  • Cell Name: chandelier pvalb GABAergic cortical interneuron (CL4023036)
    Fold Change: -0.2367
    Cell Significance Index: -4.9400
  • Cell Name: cortical interneuron (CL0008031)
    Fold Change: -0.2406
    Cell Significance Index: -5.7700

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. **Mitochondrial localization**: OTC is primarily localized to the mitochondrial matrix, where it plays a crucial role in the urea cycle and energy metabolism. 2. **Urea cycle involvement**: As a key enzyme in the urea cycle, OTC is essential for the removal of excess nitrogen from the body, which is critical for maintaining nitrogen balance and preventing toxicity. 3. **Amino acid biosynthesis**: OTC is involved in the biosynthesis of arginine and citrulline, which are essential amino acids for various cellular processes. 4. **Energy metabolism**: As a mitochondrial enzyme, OTC is also involved in energy metabolism, particularly in the context of the urea cycle, where it helps to generate ATP and NADH. 5. **Response to environmental stimuli**: OTC is responsive to various environmental stimuli, including biotin, insulin, and xenobiotics, which can affect its activity and expression. **Pathways and Functions:** OTC is involved in several key pathways, including: 1. **Urea cycle**: OTC catalyzes the conversion of carbamoyl phosphate and ornithine to citrulline, a key step in the urea cycle. 2. **Arginine biosynthesis**: OTC is involved in the biosynthesis of arginine, which is a critical amino acid for various cellular processes. 3. **Citrulline biosynthesis**: OTC is also involved in the biosynthesis of citrulline, which is an essential amino acid for various cellular processes. 4. **Energy metabolism**: As a mitochondrial enzyme, OTC is involved in energy metabolism, particularly in the context of the urea cycle, where it helps to generate ATP and NADH. 5. **Response to biotin**: OTC is responsive to biotin, a vitamin that is essential for various cellular processes, including energy metabolism and amino acid biosynthesis. **Clinical Significance:** Dysregulation of OTC has been implicated in several diseases, including: 1. **Urea cycle disorders**: Defects in OTC can lead to urea cycle disorders, which are characterized by impaired nitrogen removal and accumulation of toxic ammonia. 2. **Cancer**: OTC has been implicated in various types of cancer, including hepatocellular carcinoma and colon cancer, where it is involved in energy metabolism and amino acid biosynthesis. 3. **Neurological disorders**: OTC has also been implicated in various neurological disorders, including Alzheimer's disease and Parkinson's disease, where it is involved in energy metabolism and response to environmental stimuli. In conclusion, OTC is a critical enzyme that plays a pivotal role in the urea cycle, energy metabolism, and response to environmental stimuli. Its dysregulation has been implicated in several diseases, highlighting the importance of OTC in maintaining cellular homeostasis and preventing disease.

Genular Protein ID: 3881518410

Symbol: OTC_HUMAN

Name: Ornithine carbamoyltransferase, mitochondrial

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 6372096

Title: Structure and expression of a complementary DNA for the nuclear coded precursor of human mitochondrial ornithine transcarbamylase.

PubMed ID: 6372096

DOI: 10.1126/science.6372096

PubMed ID: 2836378

Title: Structure of the human ornithine transcarbamylase gene.

PubMed ID: 2836378

DOI: 10.1093/oxfordjournals.jbchem.a122265

PubMed ID: 14702039

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

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 15772651

Title: The DNA sequence of the human X chromosome.

PubMed ID: 15772651

DOI: 10.1038/nature03440

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: 3895227

Title: Arginine in the leader peptide is required for both import and proteolytic cleavage of a mitochondrial precursor.

PubMed ID: 3895227

DOI: 10.1073/pnas.82.15.4930

PubMed ID: 3782067

Title: Isolation and characterization of the human ornithine transcarbamylase gene: structure of the 5'-end region.

PubMed ID: 3782067

DOI: 10.1093/oxfordjournals.jbchem.a121764

PubMed ID: 8081373

Title: A novel arginine (245) to glutamine change in exon 8 of the ornithine carbamoyl transferase gene in two unrelated children presenting with late onset deficiency and showing the same enzymatic pattern.

PubMed ID: 8081373

DOI: 10.1093/hmg/3.5.831

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: 8112735

Title: Expression of four mutant human ornithine transcarbamylase genes in cultured Cos 1 cells relates to clinical phenotypes.

PubMed ID: 8112735

DOI: 10.1007/bf00210596

PubMed ID: 19318352

Title: Lysine 88 acetylation negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.

PubMed ID: 19318352

DOI: 10.1074/jbc.m901921200

PubMed ID: 9852088

Title: 1.85-A resolution crystal structure of human ornithine transcarbamoylase complexed with N-phosphonacetyl-L-ornithine. Catalytic mechanism and correlation with inherited deficiency.

PubMed ID: 9852088

DOI: 10.1074/jbc.273.51.34247

PubMed ID: 10813810

Title: Crystal structure of human ornithine transcarbamylase complexed with carbamoyl phosphate and L-norvaline at 1.9 A resolution.

PubMed ID: 10813810

DOI: 10.1002/(sici)1097-0134(20000601)39:4<271::aid-prot10>3.3.co;2-5

PubMed ID: 8364586

Title: Mutations and polymorphisms in the human ornithine transcarbamylase gene.

PubMed ID: 8364586

DOI: 10.1002/humu.1380020304

PubMed ID: 7627182

Title: Mutations and polymorphisms in the human ornithine transcarbamylase gene: mutation update addendum.

PubMed ID: 7627182

DOI: 10.1002/humu.1380050404

PubMed ID: 8544185

Title: The molecular basis of ornithine transcarbamylase deficiency: modelling the human enzyme and the effects of mutations.

PubMed ID: 8544185

DOI: 10.1136/jmg.32.9.680

PubMed ID: 3170748

Title: Characterization of point mutations in the same arginine codon in three unrelated patients with ornithine transcarbamylase deficiency.

PubMed ID: 3170748

DOI: 10.1172/jci113738

PubMed ID: 2556444

Title: An arginine to glutamine mutation in residue 109 of human ornithine transcarbamylase completely abolishes enzymatic activity in Cos1 cells.

PubMed ID: 2556444

DOI: 10.1172/jci114360

PubMed ID: 2474822

Title: Scanning detection of mutations in human ornithine transcarbamoylase by chemical mismatch cleavage.

PubMed ID: 2474822

DOI: 10.1073/pnas.86.15.5888

PubMed ID: 2347583

Title: Use of denaturing gradient gel electrophoresis for detection of mutation and prospective diagnosis in late onset ornithine transcarbamylase deficiency.

PubMed ID: 2347583

DOI: 10.1016/0888-7543(90)90537-5

PubMed ID: 1671317

Title: Improved molecular diagnostics for ornithine transcarbamylase deficiency.

PubMed ID: 1671317

PubMed ID: 1721894

Title: Fatal hyperammonemia resulting from a C-to-T mutation at a MspI site of the ornithine transcarbamylase gene.

PubMed ID: 1721894

DOI: 10.1007/bf00206063

PubMed ID: 1480464

Title: Six new mutations in the ornithine transcarbamylase gene detected by single-strand conformational polymorphism.

PubMed ID: 1480464

DOI: 10.1203/00006450-199211000-00024

PubMed ID: 8099056

Title: Single-strand conformational polymorphism and direct sequencing applied to carrier testing in families with ornithine transcarbamylase deficiency.

PubMed ID: 8099056

DOI: 10.1007/bf00217350

PubMed ID: 8019569

Title: The ornithine transcarbamylase gene: new 'private' mutations in four patients and study of a polymorphism.

PubMed ID: 8019569

DOI: 10.1002/humu.1380030325

PubMed ID: 8081398

Title: Four newly identified ornithine transcarbamylase (OTC) mutations (D126G, R129H, I172M and W332X) in Japanese male patients with early-onset OTC deficiency.

PubMed ID: 8081398

DOI: 10.1002/humu.1380030415

PubMed ID: 7951259

Title: Seven new mutations in the human ornithine transcarbamylase gene.

PubMed ID: 7951259

DOI: 10.1002/humu.1380040109

PubMed ID: 8530002

Title: A splicing mutation, a nonsense mutation (Y167X) and two missense mutations (I159T and A209V) in Spanish patients with ornithine transcarbamylase deficiency.

PubMed ID: 8530002

DOI: 10.1007/bf00197410

PubMed ID: 7474905

Title: A novel point mutation at codon 269 of the ornithine transcarbamylase (OTC) gene causing neonatal onset of OTC deficiency.

PubMed ID: 7474905

DOI: 10.1007/bf00710430

PubMed ID: 8807340

Title: Partial duplication [dup. TCAC (178)] and novel point mutations (T125M, G188R, A209V, and H302L) of the ornithine transcarbamylase gene in congenital hyperammonemia.

PubMed ID: 8807340

DOI: 10.1002/(sici)1098-1004(1996)8:1<74::aid-humu11>3.0.co;2-o

PubMed ID: 8956038

Title: Ornithine transcarbamylase deficiency: characterization of gene mutations and polymorphisms.

PubMed ID: 8956038

DOI: 10.1002/(sici)1098-1004(1996)8:4<333::aid-humu6>3.0.co;2-8

PubMed ID: 8956045

Title: A 3-base pair in-frame deletion in exon 8 (delGlu272/273) of the ornithine transcarbamylase gene in late-onset hyperammonemic coma.

PubMed ID: 8956045

DOI: 10.1002/(sici)1098-1004(1996)8:4<373::aid-humu13>3.0.co;2-#

PubMed ID: 8830175

Title: Identification of new mutations in the ornithine transcarbamylase (OTC) gene in Korean families.

PubMed ID: 8830175

DOI: 10.1007/bf01799346

PubMed ID: 9286441

Title: The ornithine transcarbamylase (OTC) gene: mutations in 50 Japanese families with OTC deficiency.

PubMed ID: 9286441

DOI: 10.1002/(sici)1096-8628(19970905)71:4<378::aid-ajmg2>3.3.co;2-9

PubMed ID: 9065786

Title: Expression, purification and kinetic characterization of wild-type human ornithine transcarbamylase and a recurrent mutant that produces 'late onset' hyperammonaemia.

PubMed ID: 9065786

DOI: 10.1042/bj3220625

PubMed ID: 9143919

Title: Ornithine transcarbamylase deficiency: ten new mutations and high proportion of de novo mutations in heterozygous females.

PubMed ID: 9143919

DOI: 10.1002/(sici)1098-1004(1997)9:5<409::aid-humu5>3.0.co;2-z

PubMed ID: 9266388

Title: Identification of 'private' mutations in patients with ornithine transcarbamylase deficiency.

PubMed ID: 9266388

DOI: 10.1023/a:1005301513465

PubMed ID: 9452024

Title: Ten novel mutations of the ornithine transcarbamylase (OTC) gene in OTC deficiency.

PubMed ID: 9452024

DOI: 10.1002/humu.1380110103

PubMed ID: 9452049

Title: Novel intragenic deletions and point mutations of the ornithine transcarbamylase gene in congenital hyperammonemia.

PubMed ID: 9452049

DOI: 10.1002/humu.1380110128

PubMed ID: 9452065

Title: Y55D mutation in ornithine transcarbamylase associated with late-onset hyperammonemia in a male.

PubMed ID: 9452065

DOI: 10.1002/humu.1380110144

PubMed ID: 10502831

Title: Identification of a cytogenetic deletion and of four novel mutations (Q69X, I172F, G188V, G197R) affecting the gene for ornithine transcarbamylase (OTC) in Spanish patients with OTC deficiency.

PubMed ID: 10502831

DOI: 10.1002/(sici)1098-1004(199910)14:4<352::aid-humu15>3.0.co;2-d

PubMed ID: 10070627

Title: Three novel and one recurrent ornithine carbamoyltransferase gene mutations in Polish patients.

PubMed ID: 10070627

DOI: 10.1023/a:1005476021549

PubMed ID: 10737985

Title: Lymphocyte mRNA analysis of the ornithine transcarbamylase gene in Italian OTCD male patients and manifesting carriers: identification of novel mutations.

PubMed ID: 10737985

DOI: 10.1002/(sici)1098-1004(200004)15:4<380::aid-humu12>3.0.co;2-q

PubMed ID: 11793483

Title: Identification of seven novel missense mutations, two splice-site mutations, two microdeletions and a polymorphic amino acid substitution in the gene for ornithine transcarbamylase (OTC) in patients with OTC deficiency.

PubMed ID: 11793483

DOI: 10.1002/humu.9011

PubMed ID: 16959974

Title: The consensus coding sequences of human breast and colorectal cancers.

PubMed ID: 16959974

DOI: 10.1126/science.1133427

Sequence Information:

  • Length: 354
  • Mass: 39935
  • Checksum: AE15B734F6E27A3B
  • Sequence:
  • MLFNLRILLN NAAFRNGHNF MVRNFRCGQP LQNKVQLKGR DLLTLKNFTG EEIKYMLWLS 
    ADLKFRIKQK GEYLPLLQGK SLGMIFEKRS TRTRLSTETG FALLGGHPCF LTTQDIHLGV 
    NESLTDTARV LSSMADAVLA RVYKQSDLDT LAKEASIPII NGLSDLYHPI QILADYLTLQ 
    EHYSSLKGLT LSWIGDGNNI LHSIMMSAAK FGMHLQAATP KGYEPDASVT KLAEQYAKEN 
    GTKLLLTNDP LEAAHGGNVL ITDTWISMGQ EEEKKKRLQA FQGYQVTMKT AKVAASDWTF 
    LHCLPRKPEE VDDEVFYSPR SLVFPEAENR KWTIMAVMVS LLTDYSPQLQ KPKF

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.