HUMAN:ACADM

Species (Taxon ID)	Homo sapiens (Human). (9606)
Gene Name(s)	ACADM
Protein Name(s)	Medium-chain specific acyl-CoA dehydrogenase, mitochondrial MCAD
External Links
UniProt	P11310
EMBL	M16827 M91432 M91421 M91422 M91423 M91425 M91426 M91427 M91428 M91429 M91430 M91431 AF251043 AK312629 AL357314 CH471059 BC005377 M60505
CCDS	CCDS44165.1 CCDS668.1
PIR	A29031
RefSeq	NP_000007.1 NP_001120800.1
UniGene	Hs.445040
PDB	1EGC 1EGD 1EGE 1T9G 2A1T
PDBsum	1EGC 1EGD 1EGE 1T9G 2A1T
ProteinModelPortal	P11310
SMR	P11310
BioGrid	106552
DIP	DIP-34281N
IntAct	P11310
MINT	MINT-3007693
STRING	9606.ENSP00000409612
DrugBank	DB03147
PhosphoSite	P11310
DMDM	113017
REPRODUCTION-2DPAGE	IPI00005040
UCD-2DPAGE	P11310
MaxQB	P11310
PaxDb	P11310
PRIDE	P11310
DNASU	34
Ensembl	ENST00000370841 ENST00000420607
GeneID	34
KEGG	hsa:34
UCSC	uc001dgw.4 uc009wbp.3
CTD	34
GeneCards	GC01P076190
GeneReviews	ACADM
HGNC	HGNC:89
HPA	HPA006198 HPA026542
MIM	201450 607008
neXtProt	NX_P11310
Orphanet	42
PharmGKB	PA24425
eggNOG	COG1960
GeneTree	ENSGT00760000119007
HOGENOM	HOG000131659
HOVERGEN	HBG000224
InParanoid	P11310
KO	K00249
OrthoDB	EOG74FF0S
PhylomeDB	P11310
TreeFam	TF105020
BioCyc	MetaCyc:HS04089-MONOMER
Reactome	REACT_116145 REACT_160 REACT_1697 REACT_1708
SABIO-RK	P11310
UniPathway	UPA00660
EvolutionaryTrace	P11310
GenomeRNAi	34
NextBio	131
PRO	PR:P11310
Proteomes	UP000005640
Bgee	P11310
CleanEx	HS_ACADM
ExpressionAtlas	P11310
Genevestigator	P11310
GO	GO:0030424 GO:0070062 GO:0005759 GO:0005739 GO:0005634 GO:0003995 GO:0050660 GO:0042802 GO:0070991 GO:0045329 GO:0019254 GO:0044255 GO:0006635 GO:0033539 GO:0051793 GO:0051791 GO:0055114 GO:0044281
Gene3D	1.10.540.10 2.40.110.10
InterPro	IPR006089 IPR006091 IPR009075 IPR013786 IPR009100
Pfam	PF00441 PF02770 PF02771
SUPFAM	SSF47203 SSF56645
PROSITE	PS00072 PS00073

Annotations

Qualifier	GO ID	GO term name	Reference	ECO ID	ECO term name	with/from	Aspect	Extension	Notes	Status
part_of	GO:0005759	mitochondrial matrix	PMID:16020546^[1]	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
part_of	GO:0031966	mitochondrial membrane	PMID:16020546^[1]	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
enables	GO:0070991	medium-chain-acyl-CoA dehydrogenase activity	PMID:3597357^[2]	ECO:0000314	direct assay evidence used in manual assertion		F	occurs_in:(UBERON:0002107)	Seeded From UniProt	complete
involved_in	GO:0033539	fatty acid beta-oxidation using acyl-CoA dehydrogenase	PMID:3597357^[2]	ECO:0000314	direct assay evidence used in manual assertion		P	occurs_in:(UBERON:0002107) has_input:(CHEBI:27540)\|occurs_in(UBERON:0002107) has_input:(CHEBI:15517)\|occurs_in(UBERON:0002107) has_input:(CHEBI:15536)\|occurs_in(UBERON:0002107) has_input:(CHEBI:15533)\|occurs_in(UBERON:0002107) has_input:(CHEBI:28493)\|occurs_in(UBERON:0002107) has_input:(CHEBI:15521)\|occurs_in(UBERON:0002107) has_input:(CHEBI:15532)\|occurs_in(UBERON:0002107) has_input:(CHEBI:15525)	Seeded From UniProt	complete
involved_in	GO:0033539	fatty acid beta-oxidation using acyl-CoA dehydrogenase	PMID:25416781^[3]	ECO:0000314	direct assay evidence used in manual assertion		P		Seeded From UniProt	complete
part_of	GO:0005634	nucleus	PMID:21630459^[4]	ECO:0007005	high throughput direct assay evidence used in manual assertion		C	part_of:(CL:0000019)	Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	GO_REF:0000054	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
enables	GO:0070991	medium-chain-acyl-CoA dehydrogenase activity	PMID:1970566^[5]	ECO:0000314	direct assay evidence used in manual assertion		F		Seeded From UniProt	complete
enables	GO:0070991	medium-chain-acyl-CoA dehydrogenase activity	PMID:19224950^[6]	ECO:0000314	direct assay evidence used in manual assertion		F		Seeded From UniProt	complete
enables	GO:0070991	medium-chain-acyl-CoA dehydrogenase activity	PMID:1902818^[7]	ECO:0000314	direct assay evidence used in manual assertion		F		Seeded From UniProt	complete
involved_in	GO:0055114	oxidation-reduction process	PMID:1902818^[7]	ECO:0000314	direct assay evidence used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0051793	medium-chain fatty acid catabolic process	PMID:1970566^[5]	ECO:0000314	direct assay evidence used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0051791	medium-chain fatty acid metabolic process	PMID:1970566^[5]	ECO:0000314	direct assay evidence used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0045329	carnitine biosynthetic process	PMID:16972171^[8]	ECO:0000315	mutant phenotype evidence used in manual assertion		P		Seeded From UniProt	complete
enables	GO:0042802	identical protein binding	PMID:19224950^[6]	ECO:0000314	direct assay evidence used in manual assertion		F		Seeded From UniProt	complete
involved_in	GO:0033539	fatty acid beta-oxidation using acyl-CoA dehydrogenase	PMID:16972171^[8]	ECO:0000315	mutant phenotype evidence used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0033539	fatty acid beta-oxidation using acyl-CoA dehydrogenase	PMID:19224950^[6]	ECO:0000314	direct assay evidence used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0033539	fatty acid beta-oxidation using acyl-CoA dehydrogenase	PMID:1902818^[7]	ECO:0000314	direct assay evidence used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0019254	carnitine metabolic process, CoA-linked	PMID:16972171^[8]	ECO:0000315	mutant phenotype evidence used in manual assertion		P		Seeded From UniProt	complete
enables	GO:0003995	acyl-CoA dehydrogenase activity	PMID:19224950^[6]	ECO:0000314	direct assay evidence used in manual assertion		F		Seeded From UniProt	complete
part_of	GO:0030424	axon	PMID:21237683^[9]	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
involved_in	GO:0006635	fatty acid beta-oxidation	PMID:2393404^[10]	ECO:0000315	mutant phenotype evidence used in manual assertion		P		Seeded From UniProt	complete
enables	GO:0003995	acyl-CoA dehydrogenase activity	PMID:2393404^[10]	ECO:0000315	mutant phenotype evidence used in manual assertion		F		Seeded From UniProt	complete
enables	GO:0070991	medium-chain-acyl-CoA dehydrogenase activity	PMID:21873635^[11]	ECO:0000318	biological aspect of ancestor evidence used in manual assertion	MGI:MGI:87867 PANTHER:PTN000098033 UniProtKB:P11310	F		Seeded From UniProt	complete
involved_in	GO:0051793	medium-chain fatty acid catabolic process	PMID:21873635^[11]	ECO:0000318	biological aspect of ancestor evidence used in manual assertion	PANTHER:PTN000098033 RGD:2012 UniProtKB:P11310	P		Seeded From UniProt	complete
involved_in	GO:0006635	fatty acid beta-oxidation	PMID:21873635^[11]	ECO:0000318	biological aspect of ancestor evidence used in manual assertion	FB:FBgn0086712 PANTHER:PTN000098033 UniProtKB:P11310	P		Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	PMID:21873635^[11]	ECO:0000318	biological aspect of ancestor evidence used in manual assertion	FB:FBgn0086712 MGI:MGI:87867 PANTHER:PTN000098033 RGD:2012 UniProtKB:P11310	C		Seeded From UniProt	complete
part_of	GO:0016607	nuclear speck	GO_REF:0000052	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	GO_REF:0000052	ECO:0000314	direct assay evidence used in manual assertion		C		Seeded From UniProt	complete
enables	GO:0003995	acyl-CoA dehydrogenase activity	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR006089	F		Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR034180	C		Seeded From UniProt	complete
involved_in	GO:0006635	fatty acid beta-oxidation	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR034180	P		Seeded From UniProt	complete
enables	GO:0016627	oxidoreductase activity, acting on the CH-CH group of donors	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR006091 InterPro:IPR009075 InterPro:IPR009100 InterPro:IPR013786 InterPro:IPR036250 InterPro:IPR037069	F		Seeded From UniProt	complete
enables	GO:0050660	flavin adenine dinucleotide binding	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR013786 InterPro:IPR037069	F		Seeded From UniProt	complete
involved_in	GO:0055114	oxidation-reduction process	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR006089 InterPro:IPR006091 InterPro:IPR009075 InterPro:IPR009100 InterPro:IPR013786 InterPro:IPR036250 InterPro:IPR037069	P		Seeded From UniProt	complete
enables	GO:0070991	medium-chain-acyl-CoA dehydrogenase activity	GO_REF:0000002	ECO:0000256	match to sequence model evidence used in automatic assertion	InterPro:IPR034180	F		Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	PMID:1731887^[12]	ECO:0000304	author statement supported by traceable reference used in manual assertion		C		Seeded From UniProt	complete
involved_in	GO:0006635	fatty acid beta-oxidation	Reactome:R-HSA-77346 Reactome:R-HSA-109341 Reactome:R-HSA-77348	ECO:0000304	author statement supported by traceable reference used in manual assertion		P		Seeded From UniProt	complete
part_of	GO:0005759	mitochondrial matrix	Reactome:R-HSA-77345 Reactome:R-HSA-77338 Reactome:R-HSA-1989745 Reactome:R-HSA-109341	ECO:0000304	author statement supported by traceable reference used in manual assertion		C		Seeded From UniProt	complete
involved_in	GO:0019216	regulation of lipid metabolic process	Reactome:R-HSA-400206	ECO:0000304	author statement supported by traceable reference used in manual assertion		P		Seeded From UniProt	complete
involved_in	GO:0055114	oxidation-reduction process	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0560	P		Seeded From UniProt	complete
part_of	GO:0005739	mitochondrion	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0496	C		Seeded From UniProt	complete
involved_in	GO:0006631	fatty acid metabolic process	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0276	P		Seeded From UniProt	complete
involved_in	GO:0006629	lipid metabolic process	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0443	P		Seeded From UniProt	complete
enables	GO:0016491	oxidoreductase activity	GO_REF:0000037	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-KW:KW-0560	F		Seeded From UniProt	complete
part_of	GO:0005759	mitochondrial matrix	GO_REF:0000039	ECO:0000322	imported manually asserted information used in automatic assertion	UniProtKB-SubCell:SL-0170	C		Seeded From UniProt	complete
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Notes

References

See Help:References for how to manage references in GONUTS.

↑ ^1.0 ^1.1 Ensenauer, R et al. (2005) Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids. J. Biol. Chem. 280 32309-16 PubMed GONUTS page
↑ ^2.0 ^2.1 Finocchiaro, G et al. (1987) Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver. J. Biol. Chem. 262 7982-9 PubMed GONUTS page
↑ Małecki, J et al. (2015) Human METTL20 is a mitochondrial lysine methyltransferase that targets the β subunit of electron transfer flavoprotein (ETFβ) and modulates its activity. J. Biol. Chem. 290 423-34 PubMed GONUTS page
↑ de Mateo, S et al. (2011) Proteomic characterization of the human sperm nucleus. Proteomics 11 2714-26 PubMed GONUTS page
↑ ^5.0 ^5.1 ^5.2 Bross, P et al. (1990) Characterization of wild-type and an active site mutant of human medium chain acyl-CoA dehydrogenase after expression in Escherichia coli. J. Biol. Chem. 265 7116-9 PubMed GONUTS page
↑ ^6.0 ^6.1 ^6.2 ^6.3 Maier, EM et al. (2009) Protein misfolding is the molecular mechanism underlying MCADD identified in newborn screening. Hum. Mol. Genet. 18 1612-23 PubMed GONUTS page
↑ ^7.0 ^7.1 ^7.2 Gregersen, N et al. (1991) Molecular characterization of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: identification of a lys329 to glu mutation in the MCAD gene, and expression of inactive mutant enzyme protein in E. coli. Hum. Genet. 86 545-51 PubMed GONUTS page
↑ ^8.0 ^8.1 ^8.2 Huidekoper, HH et al. (2006) Prolonged moderate-intensity exercise without and with L-carnitine supplementation in patients with MCAD deficiency. J. Inherit. Metab. Dis. 29 631-6 PubMed GONUTS page
↑ He, M et al. (2011) Identification and characterization of new long chain acyl-CoA dehydrogenases. Mol. Genet. Metab. 102 418-29 PubMed GONUTS page
↑ ^10.0 ^10.1 Matsubara, Y et al. (1990) Identification of a common mutation in patients with medium-chain acyl-CoA dehydrogenase deficiency. Biochem. Biophys. Res. Commun. 171 498-505 PubMed GONUTS page
↑ ^11.0 ^11.1 ^11.2 ^11.3 Gaudet, P et al. (2011) Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Brief. Bioinformatics 12 449-62 PubMed GONUTS page
↑ Zhang, ZF et al. (1992) Structural organization and regulatory regions of the human medium-chain acyl-CoA dehydrogenase gene. Biochemistry 31 81-9 PubMed GONUTS page

[PMID:16020546-1] 1.0 ^1.1 Ensenauer, R et al. (2005) Human acyl-CoA dehydrogenase-9 plays a novel role in the mitochondrial beta-oxidation of unsaturated fatty acids. J. Biol. Chem. 280 32309-16 PubMed GONUTS page

[PMID:3597357-2] 2.0 ^2.1 Finocchiaro, G et al. (1987) Purification and properties of short chain acyl-CoA, medium chain acyl-CoA, and isovaleryl-CoA dehydrogenases from human liver. J. Biol. Chem. 262 7982-9 PubMed GONUTS page

[PMID:25416781-3] Małecki, J et al. (2015) Human METTL20 is a mitochondrial lysine methyltransferase that targets the β subunit of electron transfer flavoprotein (ETFβ) and modulates its activity. J. Biol. Chem. 290 423-34 PubMed GONUTS page

[PMID:21630459-4] Mateo, S et al. (2011) Proteomic characterization of the human sperm nucleus. Proteomics 11 2714-26 PubMed GONUTS page

[PMID:1970566-5] 5.0 ^5.1 ^5.2 Bross, P et al. (1990) Characterization of wild-type and an active site mutant of human medium chain acyl-CoA dehydrogenase after expression in Escherichia coli. J. Biol. Chem. 265 7116-9 PubMed GONUTS page

[PMID:19224950-6] 6.0 ^6.1 ^6.2 ^6.3 Maier, EM et al. (2009) Protein misfolding is the molecular mechanism underlying MCADD identified in newborn screening. Hum. Mol. Genet. 18 1612-23 PubMed GONUTS page

[PMID:1902818-7] 7.0 ^7.1 ^7.2 Gregersen, N et al. (1991) Molecular characterization of medium-chain acyl-CoA dehydrogenase (MCAD) deficiency: identification of a lys329 to glu mutation in the MCAD gene, and expression of inactive mutant enzyme protein in E. coli. Hum. Genet. 86 545-51 PubMed GONUTS page

[PMID:16972171-8] 8.0 ^8.1 ^8.2 Huidekoper, HH et al. (2006) Prolonged moderate-intensity exercise without and with L-carnitine supplementation in patients with MCAD deficiency. J. Inherit. Metab. Dis. 29 631-6 PubMed GONUTS page

[PMID:21237683-9] He, M et al. (2011) Identification and characterization of new long chain acyl-CoA dehydrogenases. Mol. Genet. Metab. 102 418-29 PubMed GONUTS page

[PMID:2393404-10] 10.0 ^10.1 Matsubara, Y et al. (1990) Identification of a common mutation in patients with medium-chain acyl-CoA dehydrogenase deficiency. Biochem. Biophys. Res. Commun. 171 498-505 PubMed GONUTS page

[PMID:21873635-11] 11.0 ^11.1 ^11.2 ^11.3 Gaudet, P et al. (2011) Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Brief. Bioinformatics 12 449-62 PubMed GONUTS page

[PMID:1731887-12] Zhang, ZF et al. (1992) Structural organization and regulatory regions of the human medium-chain acyl-CoA dehydrogenase gene. Biochemistry 31 81-9 PubMed GONUTS page

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HUMAN:ACADM

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