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IVD
HPA
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  • IVD
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.

IVD
Synonyms ACAD2, IVDH
Gene descriptioni

Full gene name according to HGNC.

Isovaleryl-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 15
Cytoband q15.1
Chromosome location (bp) 40405485 - 40435947
Number of transcriptsi

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

10
Ensembl ENSG00000128928 (version 109)
Entrez gene 3712
HGNC HGNC:6186
UniProt P26440 (UniProt - Evidence at protein level)
neXtProt NX_P26440
GeneCards IVD
Antibodypedia IVD antibodies


PROTEIN FUNCTION
Protein function (UniProt)i

Useful information about the protein provided by UniProt.

Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway 1. To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA 2.... show less
Molecular function (UniProt)i

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

Oxidoreductase
Biological process (UniProt)i

Keywords assigned by UniProt to proteins because they are involved in a particular biological process.

Fatty acid metabolism, Lipid metabolism
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

Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Aug 2017]... 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)
IVD-201
ENSP00000454145
ENST00000466756
H0YNT5
[Direct mapping] Isovaleryl-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
58 aa
6.2 kDa
No 0
IVD-202
ENSP00000417256
ENST00000473112
H7C4G6
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
   SPOCTOPUS predicted membrane 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:0005739 [mitochondrion]
GO:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
307 aa
32.8 kDa
No 0
IVD-203
ENSP00000417990
ENST00000479013
A0A0S2Z4K7
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
   SPOCTOPUS predicted membrane 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:0003824 [catalytic activity]
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0004085 [butyryl-CoA dehydrogenase activity]
GO:0005739 [mitochondrion]
GO:0006552 [leucine catabolic process]
GO:0008470 [isovaleryl-CoA dehydrogenase activity]
GO:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
GO:1901565 [organonitrogen compound catabolic process]
Show all
393 aa
42.7 kDa
No 0
IVD-206
ENSP00000418397
ENST00000487418
A0A0A0MT83
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
   SPOCTOPUS predicted membrane 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:0003824 [catalytic activity]
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0004085 [butyryl-CoA dehydrogenase activity]
GO:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006552 [leucine catabolic process]
GO:0008470 [isovaleryl-CoA dehydrogenase activity]
GO:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0031966 [mitochondrial membrane]
GO:0042802 [identical protein binding]
GO:0050660 [flavin adenine dinucleotide binding]
GO:1901565 [organonitrogen compound catabolic process]
Show all
423 aa
46.3 kDa
No 0
IVD-207
ENSP00000453146
ENST00000491554
H0YLC3
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
   SPOCTOPUS predicted membrane 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:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
243 aa
26.4 kDa
No 0
IVD-210
ENSP00000453821
ENST00000558610
H0YN10
[Direct mapping] Isovaleryl-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:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
212 aa
23.4 kDa
No 0
IVD-211
ENSP00000453929
ENST00000559575
H0YNA4
[Direct mapping] Isovaleryl-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
41 aa
4.1 kDa
No 0
IVD-213
ENSP00000479359
ENST00000610693
A0A087WVD3
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
Show all
Metabolic proteins
   SCAMPI predicted membrane 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:0016491 [oxidoreductase activity]
GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors]
GO:0050660 [flavin adenine dinucleotide binding]
Show all
211 aa
23 kDa
No 0
IVD-214
ENSP00000498731
ENST00000650656
P26440
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
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:0003824 [catalytic activity]
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0004085 [butyryl-CoA dehydrogenase activity]
GO:0005515 [protein binding]
GO:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006552 [leucine catabolic process]
GO:0006629 [lipid metabolic process]
GO:0006631 [fatty acid metabolic process]
GO:0008470 [isovaleryl-CoA dehydrogenase activity]
GO:0009083 [branched-chain amino acid catabolic 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:0042802 [identical protein binding]
GO:0050660 [flavin adenine dinucleotide binding]
GO:1901565 [organonitrogen compound catabolic process]
Show all
396 aa
43.1 kDa
No 0
IVD-215
ENSP00000499074
ENST00000651168
P26440
[Direct mapping] Isovaleryl-CoA dehydrogenase, mitochondrial
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:0003824 [catalytic activity]
GO:0003995 [acyl-CoA dehydrogenase activity]
GO:0004085 [butyryl-CoA dehydrogenase activity]
GO:0005515 [protein binding]
GO:0005654 [nucleoplasm]
GO:0005739 [mitochondrion]
GO:0005759 [mitochondrial matrix]
GO:0006552 [leucine catabolic process]
GO:0006629 [lipid metabolic process]
GO:0006631 [fatty acid metabolic process]
GO:0008470 [isovaleryl-CoA dehydrogenase activity]
GO:0009083 [branched-chain amino acid catabolic 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:0042802 [identical protein binding]
GO:0050660 [flavin adenine dinucleotide binding]
GO:1901565 [organonitrogen compound catabolic process]
Show all
426 aa
46.7 kDa
No 0

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