Details for: GCDH

Gene ID: 2639

Symbol: GCDH

Ensembl ID: ENSG00000105607

Description: glutaryl-CoA dehydrogenase

Associated with

Cells (max top 100)

(Marker Score score is uniquely calculated using our advanced thresholding algorithms to reveal cell-specific gene markers)

  • Cell Name: pulmonary interstitial fibroblast (CL0002241)
    Fold Change: 1.48
    Marker Score: 1188
  • Cell Name: decidual natural killer cell, human (CL0002343)
    Fold Change: 1.09
    Marker Score: 3023
  • Cell Name: proerythroblast (CL0000547)
    Fold Change: 1.09
    Marker Score: 740
  • Cell Name: cerebral cortex GABAergic interneuron (CL0010011)
    Fold Change: 1
    Marker Score: 71718
  • Cell Name: forebrain radial glial cell (CL0013000)
    Fold Change: 1
    Marker Score: 47941.5
  • Cell Name: absorptive cell (CL0000212)
    Fold Change: 0.98
    Marker Score: 30395
  • Cell Name: pro-T cell (CL0000827)
    Fold Change: 0.97
    Marker Score: 3972.5
  • Cell Name: intestinal crypt stem cell of colon (CL0009043)
    Fold Change: 0.94
    Marker Score: 2398
  • Cell Name: tuft cell of colon (CL0009041)
    Fold Change: 0.92
    Marker Score: 476
  • Cell Name: BEST4+ intestinal epithelial cell, human (CL4030026)
    Fold Change: 0.91
    Marker Score: 432
  • Cell Name: astrocyte of the cerebral cortex (CL0002605)
    Fold Change: 0.91
    Marker Score: 19430
  • Cell Name: abnormal cell (CL0001061)
    Fold Change: 0.9
    Marker Score: 2702
  • Cell Name: kidney proximal straight tubule epithelial cell (CL1000839)
    Fold Change: 0.89
    Marker Score: 2102
  • Cell Name: neoplastic cell (CL0001063)
    Fold Change: 0.87
    Marker Score: 5294
  • Cell Name: cardiac muscle myoblast (CL0000513)
    Fold Change: 0.86
    Marker Score: 13459
  • Cell Name: transit amplifying cell (CL0009010)
    Fold Change: 0.85
    Marker Score: 4854
  • Cell Name: epithelial cell of small intestine (CL0002254)
    Fold Change: 0.85
    Marker Score: 305
  • Cell Name: erythroid progenitor cell (CL0000038)
    Fold Change: 0.81
    Marker Score: 846
  • Cell Name: plasmablast (CL0000980)
    Fold Change: 0.8
    Marker Score: 1067
  • Cell Name: cardiac endothelial cell (CL0010008)
    Fold Change: 0.8
    Marker Score: 1519
  • Cell Name: fraction A pre-pro B cell (CL0002045)
    Fold Change: 0.78
    Marker Score: 785
  • Cell Name: intestinal epithelial cell (CL0002563)
    Fold Change: 0.78
    Marker Score: 1263
  • Cell Name: kidney epithelial cell (CL0002518)
    Fold Change: 0.76
    Marker Score: 808.5
  • Cell Name: epithelial cell of nephron (CL1000449)
    Fold Change: 0.76
    Marker Score: 1593
  • Cell Name: small intestine goblet cell (CL1000495)
    Fold Change: 0.76
    Marker Score: 305
  • Cell Name: kidney cell (CL1000497)
    Fold Change: 0.76
    Marker Score: 506
  • Cell Name: early T lineage precursor (CL0002425)
    Fold Change: 0.75
    Marker Score: 570
  • Cell Name: large pre-B-II cell (CL0000957)
    Fold Change: 0.74
    Marker Score: 2023
  • Cell Name: stem cell (CL0000034)
    Fold Change: 0.74
    Marker Score: 1754
  • Cell Name: Cajal-Retzius cell (CL0000695)
    Fold Change: 0.72
    Marker Score: 371
  • Cell Name: centrilobular region hepatocyte (CL0019029)
    Fold Change: 0.71
    Marker Score: 4563
  • Cell Name: choroid plexus epithelial cell (CL0000706)
    Fold Change: 0.7
    Marker Score: 645.5
  • Cell Name: germ cell (CL0000586)
    Fold Change: 0.7
    Marker Score: 1221
  • Cell Name: L6b glutamatergic cortical neuron (CL4023038)
    Fold Change: 0.68
    Marker Score: 5864
  • Cell Name: enterocyte of epithelium of small intestine (CL1000334)
    Fold Change: 0.67
    Marker Score: 2812
  • Cell Name: brush cell (CL0002204)
    Fold Change: 0.67
    Marker Score: 608
  • Cell Name: neutrophil progenitor cell (CL0000834)
    Fold Change: 0.66
    Marker Score: 170
  • Cell Name: mesenchymal cell (CL0008019)
    Fold Change: 0.66
    Marker Score: 1123
  • Cell Name: granulocyte monocyte progenitor cell (CL0000557)
    Fold Change: 0.66
    Marker Score: 419
  • Cell Name: osteoclast (CL0000092)
    Fold Change: 0.66
    Marker Score: 328
  • Cell Name: precursor B cell (CL0000817)
    Fold Change: 0.66
    Marker Score: 434
  • Cell Name: primitive red blood cell (CL0002355)
    Fold Change: 0.66
    Marker Score: 492
  • Cell Name: midzonal region hepatocyte (CL0019028)
    Fold Change: 0.65
    Marker Score: 2811.5
  • Cell Name: early lymphoid progenitor (CL0000936)
    Fold Change: 0.64
    Marker Score: 315
  • Cell Name: respiratory epithelial cell (CL0002368)
    Fold Change: 0.64
    Marker Score: 354
  • Cell Name: mononuclear cell (CL0000842)
    Fold Change: 0.64
    Marker Score: 206
  • Cell Name: astrocyte (CL0000127)
    Fold Change: 0.63
    Marker Score: 545
  • Cell Name: early pro-B cell (CL0002046)
    Fold Change: 0.63
    Marker Score: 6370
  • Cell Name: erythroid progenitor cell, mammalian (CL0001066)
    Fold Change: 0.63
    Marker Score: 201
  • Cell Name: lymphoid lineage restricted progenitor cell (CL0000838)
    Fold Change: 0.62
    Marker Score: 375
  • Cell Name: nephron tubule epithelial cell (CL1000494)
    Fold Change: 0.62
    Marker Score: 146
  • Cell Name: cortical thymic epithelial cell (CL0002364)
    Fold Change: 0.62
    Marker Score: 2301
  • Cell Name: acinar cell of salivary gland (CL0002623)
    Fold Change: 0.62
    Marker Score: 1413
  • Cell Name: Leydig cell (CL0000178)
    Fold Change: 0.62
    Marker Score: 667
  • Cell Name: epicardial adipocyte (CL1000309)
    Fold Change: 0.62
    Marker Score: 299
  • Cell Name: L5 extratelencephalic projecting glutamatergic cortical neuron (CL4023041)
    Fold Change: 0.61
    Marker Score: 946
  • Cell Name: ovarian surface epithelial cell (CL2000064)
    Fold Change: 0.61
    Marker Score: 1648
  • Cell Name: follicular B cell (CL0000843)
    Fold Change: 0.6
    Marker Score: 654
  • Cell Name: L2/3-6 intratelencephalic projecting glutamatergic neuron (CL4023040)
    Fold Change: 0.6
    Marker Score: 37091.5
  • Cell Name: ependymal cell (CL0000065)
    Fold Change: 0.6
    Marker Score: 210
  • Cell Name: erythroblast (CL0000765)
    Fold Change: 0.6
    Marker Score: 373
  • Cell Name: epithelial cell of esophagus (CL0002252)
    Fold Change: 0.6
    Marker Score: 5143
  • Cell Name: CD8-alpha-alpha-positive, alpha-beta intraepithelial T cell (CL0000915)
    Fold Change: 0.59
    Marker Score: 839
  • Cell Name: periportal region hepatocyte (CL0019026)
    Fold Change: 0.59
    Marker Score: 3182
  • Cell Name: hepatocyte (CL0000182)
    Fold Change: 0.59
    Marker Score: 402
  • Cell Name: common myeloid progenitor (CL0000049)
    Fold Change: 0.58
    Marker Score: 154
  • Cell Name: neural crest cell (CL0011012)
    Fold Change: 0.58
    Marker Score: 619
  • Cell Name: migratory enteric neural crest cell (CL0002607)
    Fold Change: 0.58
    Marker Score: 543
  • Cell Name: adventitial cell (CL0002503)
    Fold Change: 0.57
    Marker Score: 141
  • Cell Name: kidney capillary endothelial cell (CL1000892)
    Fold Change: 0.57
    Marker Score: 178
  • Cell Name: bladder urothelial cell (CL1001428)
    Fold Change: 0.57
    Marker Score: 836
  • Cell Name: fat cell (CL0000136)
    Fold Change: 0.55
    Marker Score: 308
  • Cell Name: hematopoietic precursor cell (CL0008001)
    Fold Change: 0.55
    Marker Score: 192
  • Cell Name: common dendritic progenitor (CL0001029)
    Fold Change: 0.55
    Marker Score: 189
  • Cell Name: promyelocyte (CL0000836)
    Fold Change: 0.54
    Marker Score: 267
  • Cell Name: intestinal enteroendocrine cell (CL1001516)
    Fold Change: 0.53
    Marker Score: 426
  • Cell Name: peptic cell (CL0000155)
    Fold Change: 0.53
    Marker Score: 223
  • Cell Name: epithelial cell of proximal tubule (CL0002306)
    Fold Change: 0.53
    Marker Score: 1891
  • Cell Name: blood cell (CL0000081)
    Fold Change: 0.53
    Marker Score: 6169
  • Cell Name: caudal ganglionic eminence derived GABAergic cortical interneuron (CL4023070)
    Fold Change: 0.53
    Marker Score: 2048
  • Cell Name: kidney collecting duct intercalated cell (CL1001432)
    Fold Change: 0.53
    Marker Score: 877
  • Cell Name: hematopoietic stem cell (CL0000037)
    Fold Change: 0.53
    Marker Score: 278
  • Cell Name: Unknown (CL0000548)
    Fold Change: 0.53
    Marker Score: 383
  • Cell Name: endothelial cell of hepatic sinusoid (CL1000398)
    Fold Change: 0.52
    Marker Score: 114
  • Cell Name: cell in vitro (CL0001034)
    Fold Change: 0.52
    Marker Score: 17988
  • Cell Name: Schwann cell (CL0002573)
    Fold Change: 0.52
    Marker Score: 180
  • Cell Name: neural cell (CL0002319)
    Fold Change: 0.52
    Marker Score: 250
  • Cell Name: Unknown (CL0002371)
    Fold Change: 0.51
    Marker Score: 545.5
  • Cell Name: Bergmann glial cell (CL0000644)
    Fold Change: 0.51
    Marker Score: 209
  • Cell Name: intestinal crypt stem cell of large intestine (CL0009016)
    Fold Change: 0.51
    Marker Score: 153
  • Cell Name: placental villous trophoblast (CL2000060)
    Fold Change: 0.51
    Marker Score: 2001
  • Cell Name: mature astrocyte (CL0002627)
    Fold Change: 0.51
    Marker Score: 334
  • Cell Name: medullary thymic epithelial cell (CL0002365)
    Fold Change: 0.51
    Marker Score: 831
  • Cell Name: transit amplifying cell of small intestine (CL0009012)
    Fold Change: 0.51
    Marker Score: 210
  • Cell Name: keratocyte (CL0002363)
    Fold Change: 0.5
    Marker Score: 116
  • Cell Name: hematopoietic multipotent progenitor cell (CL0000837)
    Fold Change: 0.5
    Marker Score: 245
  • Cell Name: CD4-positive helper T cell (CL0000492)
    Fold Change: 0.5
    Marker Score: 545
  • Cell Name: neuroblast (sensu Vertebrata) (CL0000031)
    Fold Change: 0.5
    Marker Score: 314
  • Cell Name: respiratory goblet cell (CL0002370)
    Fold Change: 0.5
    Marker Score: 144
  • Cell Name: microfold cell of epithelium of small intestine (CL1000353)
    Fold Change: 0.5
    Marker Score: 119

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Other Information

**Key characteristics:** * GCDH is a multi-functional protein that is expressed in a variety of cell types. * It is found in the mitochondria of eukaryotic cells. * It is a key enzyme in the β-oxidation of fatty acids. * It is involved in the metabolism of amino acids, fatty acids, and cholesterol. * It is regulated by a variety of hormones, including insulin and glucagon. **Pathways and functions:** * The GCDH gene is involved in multiple metabolic pathways, including fatty-acyl-CoA binding, fatty-acyl-CoA biosynthetic process, fatty acid beta-oxidation using acyl-CoA dehydrogenase, and tryptophan metabolic process. * In the fatty-acyl-CoA binding pathway, GCDH binds to fatty acids and catalyzes the transfer of the acetyl group to coenzyme A. * In the fatty-acyl-CoA biosynthetic process, GCDH is involved in the formation of palmitoyl-CoA from palmitoyl-CoA esters. * In the fatty acid beta-oxidation pathway, GCDH is one of the key enzymes that catalyzes the oxidation of fatty acids to produce energy. * In the tryptophan metabolic process, GCDH is involved in the conversion of tryptophan to kynurenine. **Clinical significance:** * Mutations in the GCDH gene have been linked to a number of human diseases, including obesity, insulin resistance, and cancer. * GCDH inhibitors are being investigated as potential treatments for these diseases.

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 ChEBI 14 ChEMBL 1 ChiTaRS 1 DMDM 1 DNASU 1 DisGeNET 1 DrugBank 2 EC 2 EMBL 10 EPD 1 Ensembl 2 EvolutionaryTrace 1 ExpressionAtlas 1 GO 8 Gene3D 3 GeneCards 1 GeneReviews 1 GeneTree 1 Genevisible 1 GenomeRNAi 1 HGNC 1 HOGENOM 1 HPA 1 InParanoid 1 IntAct 1 InterPro 8 MANE-Select 1 MIM 3 MINT 1 MalaCards 1 MassIVE 1 MaxQB 1 NCBI Taxonomy 1 OMA 1 OpenTargets 1 Orphanet 1 OrthoDB 1 PANTHER 2 PDB 5 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 UniProtKB 1 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. For the full schema, download it here.