Nomenclature | Genomic Location |
Symbol | Acadm | Chromosome | X |
Name | Acyl-CoA dehydrogenase, C-4 to C-12 straight chain | Linkage map | unknown |
Species | Dracomimus familiaris | Genome Coordinates | ChrX: 63 Mbp |
Molecular Function |
This enzyme is specific for acyl chain lengths of 4 to 16.
Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor
Molecular Function Terms:
binding
carboxylic acid binding
monocarboxylic acid binding
fatty acid binding
cofactor binding
coenzyme binding
fatty-acyl-CoA binding
catalytic activity
isomerase activity
oxidoreductase activity
oxidoreductase activity, acting on the CH-CH group of donors
acyl-CoA dehydrogenase activity
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Human Disease Association |
Defects in ACADM are the cause of acyl-CoA dehydrogenase medium-chain deficiency (ACADMD)
[MIM:201450]. It is an autosomal recessive disease which causes fasting hypoglycemia, hepatic
dysfunction, and encephalopathy, often resulting in death in infancy.
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1 TTTCAATTTTCTTTCAAAACAAATTTTCAGGTGATAAGAAGCATTGCTACATGTGGTTGG
61 AGATCACAGTCTAGTCAAGCTACTGCAGCATTACCAAAAAGCAAACATGCAACTGGATTT
121 AGTTTTGAACTTACAGAAGAGCAGAAAGAATTTCAAGCTACAGCACGTAAATTTGCTAGG
181 GAGGAAATCTTACCTGCTGCGGCTGAGTATGACAGAACTGGAGAGTATCCCGTTCCTCTC
241 ATTAAAAGGGCATGGGAGCTTGGCTTGATGAATACTCACATCCCTGAAAGCTGTGGTGGC
301 CTTGGTCTTGGCACTTTTGATGCCTGCCTCATTACTGAAGAATTAGCATTTGGATGTACA
361 GGGGTTCAAACTGCCATCGAAGCAAATTCCCTGGGACAAATGCCAGTCATCATTGCAGGG
421 AATGAGCAGCAACAAAAGAAGTACCTGGGAAGAATGACAGAAGCACCCTTAATGTGTGCC
481 TACTGTGTAACAGAGCCTGGAGCTGGTTCAGATGTTGCAGGTCTAAAAACAAAAGCAGAA
541 AAGAAAGGAGACGAATATATAATTAATGGGCAAAAAATGTGGATCACCAATGGTGGCAAA
601 GCCAACTGGTATTTTTTGCTGGCTCGCACTGTTCCTGATCCCAAAGCTCCTACAAATCAA
661 GCTTTTACTGGCTTTATCGTGGAAGCAGATAGCCCTGGAATACAAATAGGCAGAAAGGAA
721 CTTAATATGGGTCAGCGTTGCTCAGATACAAGAGGGATTGTTTTCGAGGATGTAAGAGTG
781 CCCAAAGAGAATATCCTCATTGGTGAAGGAGCTGGCTTTAAAATAGCCATGGGAGCATTT
841 GATAAAACAAGGCCCCCAGTAGCAGCTGGTGCTGTAGGACTAGCACAGCGAGCCTTAGAT
901 GAAGCTACAAGGTATGCTCTGGAAAGAAAAACCTTTGGCAGGGTACTTGCAGAGCACCAA
961 GCAGTATCTTTCATGTTGGCTGAAATGGCAATGAAAGTTGAGCTTGCTCGCCTGGCT
Predicted Protein Product |
FQFSFKTNFQVIRSIATCGWRSQSSQATAALPKSKHATGFSFELTEEQKEFQATARKFAR
EEILPAAAEYDRTGEYPVPLIKRAWELGLMNTHIPESCGGLGLGTFDACLITEELAFGCT
GVQTAIEANSLGQMPVIIAGNEQQQKKYLGRMTEAPLMCAYCVTEPGAGSDVAGLKTKAE
KKGDEYIINGQKMWITNGGKANWYFLLARTVPDPKAPTNQAFTGFIVEADSPGIQIGRKE
LNMGQRCSDTRGIVFEDVRVPKENILIGEGAGFKIAMGAFDKTRPPVAAGAVGLAQRALD
EATRYALERKTFGRVLAEHQAVSFMLAEMAMKVELARLA
Protein Alignment to Mouse |
sp|P45952|ACADM_MOUSE Medium-chain specific acyl-CoA dehydrogenase,
mitochondrial OS=Mus musculus GN=Acadm PE=1 SV=1
MGI:87867 Acadm acyl-Coenzyme A dehydrogenase, medium chain (Chr 3)
Length = 421
Score = 1432 (509.1 bits), Expect = 2.6e-147, P = 2.6e-147
Identities = 270/336 (80%), Positives = 304/336 (90%)
Query: 4 SFKTNFQVIRSIATCGWRSQSSQATAALPKSKHATGFSFELTEEQKEFQATARKFAREEI 63
+F+ +V+RS++ R+Q S+A K + GFSFELTE+QKEFQATARKFAREEI
Sbjct: 4 AFRRGCRVLRSVSHFECRTQHSKAAH---KQEPGLGFSFELTEQQKEFQATARKFAREEI 60
Query: 64 LPAAAEYDRTGEYPVPLIKRAWELGLMNTHIPESCGGLGLGTFDACLITEELAFGCTGVQ 123
+P A EYD++GEYP PLIKRAWELGL+N HIPESCGGLGLGTFDACLITEELA+GCTGVQ
Sbjct: 61 IPVAPEYDKSGEYPFPLIKRAWELGLINAHIPESCGGLGLGTFDACLITEELAYGCTGVQ 120
Query: 124 TAIEANSLGQMPVIIAGNEQQQKKYLGRMTEAPLMCAYCVTEPGAGSDVAGLKTKAEKKG 183
TAIEANSLGQMPVI+AGN+QQ+KKYLGRMTE P+MCAYCVTEP AGSDVA +KTKAEKKG
Sbjct: 121 TAIEANSLGQMPVILAGNDQQKKKYLGRMTEQPMMCAYCVTEPSAGSDVAAIKTKAEKKG 180
Query: 184 DEYIINGQKMWITNGGKANWYFLLARTVPDPKAPTNQAFTGFIVEADSPGIQIGRKELNM 243
DEY+INGQKMWITNGGKANWYFLLAR+ PDPK P ++AFTGFIVEAD+PGI IG+KELNM
Sbjct: 181 DEYVINGQKMWITNGGKANWYFLLARSNPDPKVPASKAFTGFIVEADTPGIHIGKKELNM 240
Query: 244 GQRCSDTRGIVFEDVRVPKENILIGEGAGFKIAMGAFDKTRPPVAAGAVGLAQRALDEAT 303
GQRCSDTRGI FEDVRVPKEN+LIGEGAGFKIAMGAFD+TRP VAAGAVGLAQRALDEAT
Sbjct: 241 GQRCSDTRGIAFEDVRVPKENVLIGEGAGFKIAMGAFDRTRPTVAAGAVGLAQRALDEAT 300
Query: 304 RYALERKTFGRVLAEHQAVSFMLAEMAMKVELARLA 339
+YAL+RKTFG++L EHQ VSF+LAEMAMKVELARL+
Sbjct: 301 KYALDRKTFGKLLVEHQGVSFLLAEMAMKVELARLS 336
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