Details for: GCDH

Gene ID: 2639

Symbol: GCDH

Ensembl ID: ENSG00000105607

Description: glutaryl-CoA dehydrogenase

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: polychromatophilic erythroblast (CL0000550)
    Fold Change: 90.0060
    Cell Significance Index: -14.0000
  • Cell Name: hematopoietic oligopotent progenitor cell (CL0002032)
    Fold Change: 57.0880
    Cell Significance Index: -14.4800
  • Cell Name: mucosal type mast cell (CL0000485)
    Fold Change: 40.9101
    Cell Significance Index: -16.6200
  • Cell Name: orthochromatic erythroblast (CL0000552)
    Fold Change: 14.4652
    Cell Significance Index: -17.8400
  • Cell Name: CD8-alpha-beta-positive, alpha-beta intraepithelial T cell (CL0000796)
    Fold Change: 5.6442
    Cell Significance Index: -15.1200
  • Cell Name: stromal cell of bone marrow (CL0010001)
    Fold Change: 4.9264
    Cell Significance Index: -19.4400
  • Cell Name: CD8-positive, alpha-beta regulatory T cell (CL0000795)
    Fold Change: 4.5841
    Cell Significance Index: -14.0800
  • Cell Name: epidermal Langerhans cell (CL0002457)
    Fold Change: 2.9882
    Cell Significance Index: -6.5400
  • Cell Name: colon goblet cell (CL0009039)
    Fold Change: 2.5706
    Cell Significance Index: 254.2900
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 1.4444
    Cell Significance Index: 234.9100
  • Cell Name: pro-T cell (CL0000827)
    Fold Change: 1.2271
    Cell Significance Index: 31.3500
  • Cell Name: gut absorptive cell (CL0000677)
    Fold Change: 1.1608
    Cell Significance Index: 69.6900
  • Cell Name: epidermal cell (CL0000362)
    Fold Change: 1.1089
    Cell Significance Index: 2.5000
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.9960
    Cell Significance Index: 197.6500
  • Cell Name: epithelial cell of stomach (CL0002178)
    Fold Change: 0.5053
    Cell Significance Index: 58.8900
  • Cell Name: basal epithelial cell of tracheobronchial tree (CL0002329)
    Fold Change: 0.4648
    Cell Significance Index: 12.9900
  • Cell Name: conjunctival epithelial cell (CL1000432)
    Fold Change: 0.4053
    Cell Significance Index: 5.5300
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 0.3939
    Cell Significance Index: 355.6700
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 0.3876
    Cell Significance Index: 18.0700
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.3736
    Cell Significance Index: 24.1100
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 0.3197
    Cell Significance Index: 64.1300
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 0.3105
    Cell Significance Index: 21.4700
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.2889
    Cell Significance Index: 15.0100
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.2745
    Cell Significance Index: 149.8900
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.2593
    Cell Significance Index: 7.4700
  • Cell Name: intermediate cell of urothelium (CL4030055)
    Fold Change: 0.2485
    Cell Significance Index: 44.7900
  • Cell Name: stromal cell of ovary (CL0002132)
    Fold Change: 0.2430
    Cell Significance Index: 33.3700
  • Cell Name: obsolete caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: 0.2255
    Cell Significance Index: 80.8700
  • Cell Name: basal cell of urothelium (CL1000486)
    Fold Change: 0.2149
    Cell Significance Index: 26.4300
  • Cell Name: paneth cell of epithelium of small intestine (CL1000343)
    Fold Change: 0.2082
    Cell Significance Index: 4.5100
  • Cell Name: skeletal muscle myoblast (CL0000515)
    Fold Change: 0.1656
    Cell Significance Index: 1.8000
  • Cell Name: tonsil germinal center B cell (CL2000006)
    Fold Change: 0.1597
    Cell Significance Index: 18.8300
  • Cell Name: GABAergic interneuron (CL0011005)
    Fold Change: 0.1592
    Cell Significance Index: 110.0900
  • Cell Name: odontoblast (CL0000060)
    Fold Change: 0.1477
    Cell Significance Index: 18.9400
  • Cell Name: hair follicular keratinocyte (CL2000092)
    Fold Change: 0.1392
    Cell Significance Index: 61.5600
  • Cell Name: enteroendocrine cell of colon (CL0009042)
    Fold Change: 0.1235
    Cell Significance Index: 23.5000
  • Cell Name: luminal adaptive secretory precursor cell of mammary gland (CL4033057)
    Fold Change: 0.0995
    Cell Significance Index: 4.6800
  • Cell Name: lactocyte (CL0002325)
    Fold Change: 0.0973
    Cell Significance Index: 12.5700
  • Cell Name: progenitor cell of mammary luminal epithelium (CL0009116)
    Fold Change: 0.0918
    Cell Significance Index: 6.8400
  • Cell Name: basal cell of prostate epithelium (CL0002341)
    Fold Change: 0.0831
    Cell Significance Index: 2.2600
  • Cell Name: enterocyte of epithelium of large intestine (CL0002071)
    Fold Change: 0.0470
    Cell Significance Index: 2.1300
  • Cell Name: mesenchymal cell (CL0008019)
    Fold Change: 0.0418
    Cell Significance Index: 0.7000
  • Cell Name: transit amplifying cell of colon (CL0009011)
    Fold Change: 0.0365
    Cell Significance Index: 1.1700
  • Cell Name: retinal progenitor cell (CL0002672)
    Fold Change: 0.0364
    Cell Significance Index: 2.0400
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: 0.0304
    Cell Significance Index: 2.3400
  • Cell Name: sebum secreting cell (CL0000317)
    Fold Change: 0.0298
    Cell Significance Index: 2.1100
  • Cell Name: fibroblast of dermis (CL0002551)
    Fold Change: 0.0284
    Cell Significance Index: 0.6000
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: 0.0208
    Cell Significance Index: 0.7300
  • Cell Name: eye photoreceptor cell (CL0000287)
    Fold Change: 0.0203
    Cell Significance Index: 1.2800
  • Cell Name: pigmented epithelial cell (CL0000529)
    Fold Change: 0.0201
    Cell Significance Index: 37.8500
  • Cell Name: lens epithelial cell (CL0002224)
    Fold Change: 0.0146
    Cell Significance Index: 22.5300
  • Cell Name: anterior lens cell (CL0002223)
    Fold Change: 0.0106
    Cell Significance Index: 19.4800
  • Cell Name: pancreatic acinar cell (CL0002064)
    Fold Change: 0.0102
    Cell Significance Index: 1.7400
  • Cell Name: non-pigmented ciliary epithelial cell (CL0002304)
    Fold Change: 0.0071
    Cell Significance Index: 4.5400
  • Cell Name: skeletal muscle fibroblast (CL0011027)
    Fold Change: 0.0059
    Cell Significance Index: 0.0400
  • Cell Name: secondary lens fiber (CL0002225)
    Fold Change: -0.0038
    Cell Significance Index: -5.1700
  • Cell Name: kidney loop of Henle cortical thick ascending limb epithelial cell (CL1001109)
    Fold Change: -0.0055
    Cell Significance Index: -4.0400
  • Cell Name: pulmonary alveolar epithelial cell (CL0000322)
    Fold Change: -0.0062
    Cell Significance Index: -4.7200
  • Cell Name: ciliary muscle cell (CL1000443)
    Fold Change: -0.0122
    Cell Significance Index: -5.5500
  • Cell Name: kidney cell (CL1000497)
    Fold Change: -0.0172
    Cell Significance Index: -0.1400
  • Cell Name: pancreatic A cell (CL0000171)
    Fold Change: -0.0263
    Cell Significance Index: -19.5000
  • Cell Name: pigmented ciliary epithelial cell (CL0002303)
    Fold Change: -0.0277
    Cell Significance Index: -4.0200
  • Cell Name: preadipocyte (CL0002334)
    Fold Change: -0.0297
    Cell Significance Index: -0.5800
  • Cell Name: cortical cell of adrenal gland (CL0002097)
    Fold Change: -0.0323
    Cell Significance Index: -0.8600
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: -0.0337
    Cell Significance Index: -3.4400
  • Cell Name: type B pancreatic cell (CL0000169)
    Fold Change: -0.0341
    Cell Significance Index: -19.2500
  • Cell Name: intestinal crypt stem cell of small intestine (CL0009017)
    Fold Change: -0.0352
    Cell Significance Index: -0.7500
  • Cell Name: pancreatic PP cell (CL0002275)
    Fold Change: -0.0352
    Cell Significance Index: -22.0000
  • Cell Name: granulosa cell (CL0000501)
    Fold Change: -0.0356
    Cell Significance Index: -0.9400
  • Cell Name: dopaminergic neuron (CL0000700)
    Fold Change: -0.0496
    Cell Significance Index: -14.2600
  • Cell Name: glycinergic neuron (CL1001509)
    Fold Change: -0.0545
    Cell Significance Index: -2.8600
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: -0.0625
    Cell Significance Index: -1.6700
  • Cell Name: mesonephric nephron tubule epithelial cell (CL1000022)
    Fold Change: -0.0712
    Cell Significance Index: -2.4800
  • Cell Name: forebrain neuroblast (CL1000042)
    Fold Change: -0.0836
    Cell Significance Index: -5.1400
  • Cell Name: centrilobular region hepatocyte (CL0019029)
    Fold Change: -0.0843
    Cell Significance Index: -1.4200
  • Cell Name: pancreatic ductal cell (CL0002079)
    Fold Change: -0.0895
    Cell Significance Index: -10.2500
  • Cell Name: pancreatic D cell (CL0000173)
    Fold Change: -0.1062
    Cell Significance Index: -22.3700
  • Cell Name: indirect pathway medium spiny neuron (CL4023029)
    Fold Change: -0.1216
    Cell Significance Index: -5.3800
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: -0.1575
    Cell Significance Index: -1.6300
  • Cell Name: direct pathway medium spiny neuron (CL4023026)
    Fold Change: -0.1598
    Cell Significance Index: -6.0500
  • Cell Name: kidney loop of Henle descending limb epithelial cell (CL1001021)
    Fold Change: -0.1610
    Cell Significance Index: -12.7500
  • Cell Name: smooth muscle cell of sphincter of pupil (CL0002243)
    Fold Change: -0.1650
    Cell Significance Index: -17.1800
  • Cell Name: umbrella cell of urothelium (CL4030056)
    Fold Change: -0.1672
    Cell Significance Index: -1.5400
  • Cell Name: hippocampal granule cell (CL0001033)
    Fold Change: -0.1820
    Cell Significance Index: -12.2400
  • Cell Name: CD4-positive, alpha-beta memory T cell, CD45RO-positive (CL0001204)
    Fold Change: -0.1859
    Cell Significance Index: -5.4600
  • Cell Name: cerebellar granule cell (CL0001031)
    Fold Change: -0.2217
    Cell Significance Index: -3.8000
  • Cell Name: intestinal tuft cell (CL0019032)
    Fold Change: -0.2363
    Cell Significance Index: -14.4900
  • Cell Name: Purkinje cell (CL0000121)
    Fold Change: -0.2644
    Cell Significance Index: -5.7900
  • Cell Name: retinal rod cell (CL0000604)
    Fold Change: -0.2718
    Cell Significance Index: -3.2400
  • Cell Name: BEST4+ enteroycte (CL4030026)
    Fold Change: -0.2794
    Cell Significance Index: -4.2100
  • Cell Name: lung endothelial cell (CL1001567)
    Fold Change: -0.3028
    Cell Significance Index: -15.7800
  • Cell Name: peg cell (CL4033014)
    Fold Change: -0.3086
    Cell Significance Index: -7.1300
  • Cell Name: cardiac muscle cell (CL0000746)
    Fold Change: -0.3109
    Cell Significance Index: -4.5900
  • Cell Name: periportal region hepatocyte (CL0019026)
    Fold Change: -0.3164
    Cell Significance Index: -4.6700
  • Cell Name: enteroendocrine cell of small intestine (CL0009006)
    Fold Change: -0.3192
    Cell Significance Index: -7.9800
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: -0.3267
    Cell Significance Index: -8.7400
  • Cell Name: kidney epithelial cell (CL0002518)
    Fold Change: -0.3483
    Cell Significance Index: -10.2600
  • Cell Name: microcirculation associated smooth muscle cell (CL0008035)
    Fold Change: -0.3542
    Cell Significance Index: -2.9800
  • Cell Name: skeletal muscle fiber (CL0008002)
    Fold Change: -0.4011
    Cell Significance Index: -10.3100
  • Cell Name: L6b glutamatergic cortical neuron (CL4023038)
    Fold Change: -0.4145
    Cell Significance Index: -13.5700

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:** GCDH is a mitochondrial enzyme that belongs to the dehydrogenase family. It is a heterodimeric complex composed of two subunits, GCDH1 and GCDH2, which are encoded by separate genes. The enzyme exhibits a high degree of specificity, with a preference for glutaryl-CoA as its substrate. GCDH is also known to interact with various co-factors, including flavin adenine dinucleotide (FAD), which is essential for its catalytic activity. **Pathways and Functions:** GCDH is involved in several critical metabolic pathways, including: 1. **Fatty acid beta-oxidation using acyl-CoA dehydrogenase**: GCDH works in conjunction with acyl-CoA dehydrogenase to facilitate the breakdown of fatty acids in the mitochondria. 2. **Lysine catabolism**: GCDH is responsible for the degradation of lysine, an essential amino acid, into succinyl-CoA and glutamate. 3. **Tryptophan metabolic process**: GCDH is also involved in the catabolism of tryptophan, another essential amino acid, into various intermediates. 4. **Glutaryl-CoA dehydrogenase activity**: GCDH catalyzes the conversion of glutaryl-CoA to succinyl-CoA, a critical step in the metabolism of branched-chain amino acids. **Clinical Significance:** Dysregulation of GCDH has been implicated in various diseases, including: 1. **Neurodegenerative disorders**: Mutations in the GCDH1 gene have been associated with neurodegenerative diseases, such as adrenoleukodystrophy and subacute sclerosing panencephalitis. 2. **Mitochondrial disorders**: GCDH deficiency has been linked to mitochondrial disorders, which are characterized by impaired energy metabolism and cellular dysfunction. 3. **Cancer**: Alterations in GCDH expression have been observed in various types of cancer, including colorectal cancer and lymphoma. In conclusion, GCDH is a vital enzyme that plays a crucial role in various metabolic pathways. Its dysregulation has been implicated in several diseases, highlighting the importance of understanding its function and regulation in the context of human health and disease. Further research is necessary to elucidate the mechanisms underlying GCDH-mediated metabolic processes and to develop therapeutic strategies for the treatment of diseases associated with GCDH dysfunction.

Genular Protein ID: 1484042796

Symbol: GCDH_HUMAN

Name: Glutaryl-CoA dehydrogenase, mitochondrial

UniProtKB Accession Codes:

Q92947 A8K2Z2 O14719

Database IDs:

AGR 1 AlphaFoldDB 1 Antibodypedia 1 BRENDA 1 Bgee 1 BindingDB 1 BioGRID 1 BioGRID-ORCS 1 BioMuta 1 CCDS 1 CDD 1 CTD 1 ChEBI 14 ChEMBL 1 ChiTaRS 1 DMDM 1 DNASU 1 DisGeNET 1 DrugBank 2 EC 2 EMBL 10 Ensembl 2 EvolutionaryTrace 1 ExpressionAtlas 1 GO 8 Gene3D 3 GeneCards 1 GeneReviews 1 GeneTree 1 GenomeRNAi 1 HGNC 1 HOGENOM 1 HPA 1 InParanoid 1 IntAct 1 InterPro 9 KEGG 1 MANE-Select 1 MIM 3 MINT 1 MalaCards 1 MassIVE 1 NCBI Taxonomy 1 OMA 1 OpenTargets 1 Orphanet 1 OrthoDB 1 PANTHER 2 PDB 4 PDBsum 4 PIR 2 PRO 1 PROSITE 2 PathwayCommons 1 PaxDb 1 PeptideAtlas 1 Pfam 3 PharmGKB 1 Pharos 1 PhosphoSitePlus 1 PhylomeDB 1 Proteomes 1 ProteomicsDB 2 Pumba 1 RNAct 1 Reactome 1 RefSeq 2 Rhea 3 SABIO-RK 1 SMR 1 STRING 1 SUPFAM 2 SignaLink 1 SwissPalm 1 TreeFam 1 UCSC 1 UniPathway 2 VEuPathDB 1 eggNOG 1 iPTMnet 1 jPOST 1 neXtProt 1

Citations:

PubMed ID: 1438360

Title: Pork and human cDNAs encoding glutaryl-CoA dehydrogenase.

PubMed ID: 1438360

PubMed ID: 8541831

Title: Cloning of glutaryl-CoA dehydrogenase cDNA, and expression of wild type and mutant enzymes in Escherichia coli.

PubMed ID: 8541831

DOI: 10.1093/hmg/4.9.1493

PubMed ID: 9600243

Title: The human glutaryl-CoA dehydrogenase gene: report of intronic sequences and of 13 novel mutations causing glutaric aciduria type I.

PubMed ID: 9600243

DOI: 10.1007/s004390050720

PubMed ID: 14702039

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

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 15057824

Title: The DNA sequence and biology of human chromosome 19.

PubMed ID: 15057824

DOI: 10.1038/nature02399

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: 6423663

Title: Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients.

PubMed ID: 6423663

DOI: 10.1172/jci111271

PubMed ID: 9711871

Title: Glutaryl-CoA dehydrogenase mutations in glutaric acidemia (type I): review and report of thirty novel mutations.

PubMed ID: 9711871

DOI: 10.1002/(sici)1098-1004(1998)12:3<141::aid-humu1>3.0.co;2-k

PubMed ID: 17176108

Title: Kinetic mechanism of glutaryl-CoA dehydrogenase.

PubMed ID: 17176108

DOI: 10.1021/bi0609016

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

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: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 15274622

Title: Crystal structures of human glutaryl-CoA dehydrogenase with and without an alternate substrate: structural bases of dehydrogenation and decarboxylation reactions.

PubMed ID: 15274622

DOI: 10.1021/bi049290c

PubMed ID: 18020372

Title: The effect of a Glu370Asp mutation in glutaryl-CoA dehydrogenase on proton transfer to the dienolate intermediate.

PubMed ID: 18020372

DOI: 10.1021/bi7009597

PubMed ID: 8900227

Title: Gene structure and mutations of glutaryl-coenzyme A dehydrogenase: impaired association of enzyme subunits that is due to an A421V substitution causes glutaric acidemia type I in the Amish.

PubMed ID: 8900227

PubMed ID: 8900228

Title: Glutaric aciduria type I in the Arab and Jewish communities in Israel.

PubMed ID: 8900228

PubMed ID: 14707522

Title: Severe phenotype despite high residual glutaryl-CoA dehydrogenase activity: a novel mutation in a Turkish patient with glutaric aciduria type I.

PubMed ID: 14707522

DOI: 10.1023/b:boli.0000005604.90621.e2

PubMed ID: 18775954

Title: Disease-causing missense mutations affect enzymatic activity, stability and oligomerization of glutaryl-CoA dehydrogenase (GCDH).

PubMed ID: 18775954

DOI: 10.1093/hmg/ddn284

PubMed ID: 24973495

Title: Molecular analysis of Cypriot patients with Glutaric aciduria type I: Identification of two novel mutations.

PubMed ID: 24973495

DOI: 10.1016/j.clinbiochem.2014.06.017

Sequence Information:

  • Length: 438
  • Mass: 48127
  • Checksum: 415B8D510027BB63
  • Sequence:
    • MALRGVSVRL LSRGPGLHVL RTWVSSAAQT EKGGRTQSQL AKSSRPEFDW QDPLVLEEQL 
TTDEILIRDT FRTYCQERLM PRILLANRNE VFHREIISEM GELGVLGPTI KGYGCAGVSS 
VAYGLLAREL ERVDSGYRSA MSVQSSLVMH PIYAYGSEEQ RQKYLPQLAK GELLGCFGLT 
EPNSGSDPSS METRAHYNSS NKSYTLNGTK TWITNSPMAD LFVVWARCED GCIRGFLLEK 
GMRGLSAPRI QGKFSLRASA TGMIIMDGVE VPEENVLPGA SSLGGPFGCL NNARYGIAWG 
VLGASEFCLH TARQYALDRM QFGVPLARNQ LIQKKLADML TEITLGLHAC LQLGRLKDQD 
KAAPEMVSLL KRNNCGKALD IARQARDMLG GNGISDEYHV IRHAMNLEAV NTYEGTHDIH 
ALILGRAITG IQAFTASK

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.