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GCDH
HPA
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  • GCDH
PROTEIN SUMMARY GENE INFORMATION RNA DATA ANTIBODY DATA
GENERAL INFORMATIONi

General description of the gene and the encoded protein(s) using information from HGNC and Ensembl, as well as predictions made by the Human Protein Atlas project.

Gene namei

Official gene symbol, which is typically a short form of the gene name, according to HGNC.

GCDH
Synonyms ACAD5, GCD
Gene descriptioni

Full gene name according to HGNC.

Glutaryl-CoA dehydrogenase
Protein classi

Assigned HPA protein class(es) for the encoded protein(s).

Disease related genes
Enzymes
Human disease related genes
Metabolic proteins
Potential drug targets
Predicted locationi

All transcripts of all genes have been analyzed regarding the location(s) of corresponding protein based on prediction methods for signal peptides and transmembrane regions.

  • Genes with at least one transcript predicted to encode a secreted protein, according to prediction methods or to UniProt location data, have been further annotated and classified with the aim to determine if the corresponding protein(s) are secreted or actually retained in intracellular locations or membrane-attached.

  • Remaining genes, with no transcript predicted to encode a secreted protein, will be assigned the prediction-based location(s).

The annotated location overrules the predicted location, so that a gene encoding a predicted secreted protein that has been annotated as intracellular will have intracellular as the final location.

Intracellular
Protein evidence Evidence at protein level (all genes)
GENE INFORMATIONi

Gene information from Ensembl and Entrez, as well as links to available gene identifiers are displayed here. Information was retrieved from Ensembl if not indicated otherwise.

Chromosome 19
Cytoband p13.13
Chromosome location (bp) 12891160 - 12914207
Number of transcriptsi

Number of protein-coding transcripts from the gene as defined by Ensembl.

7
Ensembl ENSG00000105607 (version 109)
Entrez gene 2639
HGNC HGNC:4189
UniProt Q92947 (UniProt - Evidence at protein level)
neXtProt NX_Q92947
GeneCards GCDH
Antibodypedia GCDH antibodies


PROTEIN FUNCTION
Protein function (UniProt)i

Useful information about the protein provided by UniProt.

Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.... show less
Molecular function (UniProt)i

Keywords assigned by UniProt to proteins due to their particular molecular function.

Oxidoreductase
Ligand (UniProt)i

Keywords assigned by UniProt to proteins because they bind, are associated with, or whose activity is dependent of some molecule.

FAD, Flavoprotein
Gene summary (Entrez)i

Useful information about the gene from Entrez

The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family. It catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. The enzyme exists in the mitochondrial matrix as a homotetramer of 45-kD subunits. Mutations in this gene result in the metabolic disorder glutaric aciduria type 1, which is also known as glutaric acidemia type I. Alternative splicing of this gene results in multiple transcript variants. A related pseudogene has been identified on chromosome 12. [provided by RefSeq, Mar 2013]... show less
PROTEIN INFORMATIONi

The protein information section displays alternative protein-coding transcripts (splice variants) encoded by this gene according to the Ensembl database.

The Splice variant identifier links to the Ensembl website protein summary for the selected splice variant. The data in the Swissprot and TrEMBL columns links to corresponding pages in the UniProt database.

The protein classes assigned to this protein are shown if expanding the data in the protein class column. Parent protein classes are in bold font and subclasses are listed under the parent class.

The length of the protein (amino acid residues according to Ensembl), molecular mass (kDalton), predicted signal peptide and number of predicted transmembrane region(s) according to in-house majority decision methods based on sets of predictors are also reported.
Splice variant SwissProt TrEMBL Protein class Gene ontology Length & mass Signal peptide
(predicted)
Transmembrane regions
(predicted)
GCDH-201
ENSP00000222214
ENST00000222214
Q92947
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
A0A024R7F9
[Target identity:100%; Query identity:100%] Glutaryl-Coenzyme A dehydrogenase, isoform CRA_a
Show all
Enzymes
   ENZYME proteins
   Oxidoreductases
Metabolic proteins
   SPOCTOPUS predicted membrane proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Disease related genes
Potential drug targets
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Mapped to neXtProt
   neXtProt - Evidence at protein level
Protein evidence (Kim et al 2014)
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000062 [fatty-acyl-CoA binding]
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0004361 [glutaryl-CoA dehydrogenase activity]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006568 [tryptophan metabolic process]
GO:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0033539 [fatty acid beta-oxidation using acyl-CoA dehydrogenase]
GO:0046949 [fatty-acyl-CoA biosynthetic process]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
438 aa
48.1 kDa
No 0
GCDH-205
ENSP00000468584
ENST00000587072
K7ES74
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0005739 [mitochondrion]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
131 aa
14.5 kDa
No 0
GCDH-208
ENSP00000465770
ENST00000588905
K7EKT3
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0005739 [mitochondrion]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
164 aa
18.4 kDa
No 0
GCDH-209
ENSP00000465618
ENST00000589039
K7EKH1
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
148 aa
16.7 kDa
No 0
GCDH-211
ENSP00000468625
ENST00000590472
K7ESA6
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
Show all
115 aa
12.6 kDa
No 0
GCDH-215
ENSP00000467735
ENST00000591050
K7EQ99
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
Show all
102 aa
10.8 kDa
No 0
GCDH-216
ENSP00000466845
ENST00000591470
Q92947
[Direct mapping] Glutaryl-CoA dehydrogenase, mitochondrial
Show all
A0A024R7F9
[Target identity:100%; Query identity:100%] Glutaryl-Coenzyme A dehydrogenase, isoform CRA_a
Show all
Enzymes
   ENZYME proteins
   Oxidoreductases
Metabolic proteins
   SPOCTOPUS predicted membrane proteins
Predicted intracellular proteins
   Intracellular proteins predicted by MDM and MDSEC
Disease related genes
Potential drug targets
Human disease related genes
   Congenital disorders of metabolism
   Congenital disorders of amino acid metabolism
Mapped to neXtProt
   neXtProt - Evidence at protein level
Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000062 [fatty-acyl-CoA binding]
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0004361 [glutaryl-CoA dehydrogenase activity]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006568 [tryptophan metabolic process]
GO:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0033539 [fatty acid beta-oxidation using acyl-CoA dehydrogenase]
GO:0046949 [fatty-acyl-CoA biosynthetic process]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
438 aa
48.1 kDa
No 0

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