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Gene Model: Acads

NomenclatureGenomic Location
SymbolAcadsChromosome1
NameAcyl-CoA dehydrogenase,
C-2 to C-3 short chain		Linkage mapunknown
SpeciesDracomimus familiarisGenome CoordinatesChr1: 8 Mbp

Molecular Function

Butanoyl-CoA + acceptor = 2-butenoyl-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

Human Disease Association

Defects in ACADS are the cause of acyl-CoA dehydrogenase short-chain deficiency (ACADSD) [MIM:201470]. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults.

Predicted Transcript
     1 ATAGGGAAGGCCTATTGGAGCATGGTGGAGCCCCCTTCTCGGGAGGCTTTGAAGCAGAGG
    61 CAGGCTGGCGTGCGGCGGCTGCACACAGTGTATCAAGCGGTAGACCTGCCAGAGACCCAC
   121 CTGATGCTGCGCCAGACCTGCCGAGACTTTGCCGAAAAGGAGCTGGCTCCCATTGCGGCC
   181 CAGCTGGATCGGGAGCACCGCTTCCCAGCCGAACAGGTGAAGAAGATGGGGAGTTTGGGG
   241 CTCCTGGCGATGGATGTCCCGGAGACATATGGAGGAGCTGGGCTTGATTACTTGGCCTAT
   301 TCCATCGCTGTGGAAGAGATCAGCCGAGGCTGTGCCTCCACCGGTGTCATCATGAGTGTC
   361 AACAATACCCTCTATTTAGGACCGGTGTTGAAGTATGGGTCTGAAGAACAGAAGCACAAG
   421 TGGATCACTCCCTTCACTAATGGGGACAAAATTGGATGCTTTGCCCTCAGTGAACCAGGC
   481 AATGGCAGCGACGCAGGAGCGGCTTCCACGGTGGCACGGCTTGTTGGCGACGAATGGGTC
   541 TTGAACGGGACCAAAGCCTGGATCACCAACGCCTGGGATGCTGCTGCGACGGTTGTGTTT
   601 GCCACTACGGACAAGGGCCTGAAACACAAGGGCATCAGTGCCTTCCTTGTGCCCATGCCA
   661 ACCCCCGGCTTGTCTCTGGGAAAGAAGGAAGACAAGATGGGCATCAGAGCCTCCTCCACG
   721 GCTAACCTGATCTTTGAAGACTGCCGCATCCCCAAGGACAACCTCCTGGGAGAGGAAGGC
   781 ATGGGCTTCAAGATTGCTATGCAAACCCTGGACTCTGGTCGGATTGGCATTGCCTCCCAG
   841 GCCTTAGGAATAGCCCAGGCAGCGCTGGACTGTGCGGTGGATTACGCCGAGAAGAGGATG
   901 GCCTTTGGAGCGCCCATCACCAAGTTCCAGGCAATTCAGTTTAAGCTGGCCGATATGGCC
   961 CTGGCCCTGGAGAGTGCCCGCCTGCTGACCTGGAGGGCGGCCATGTTGAAGGACAACGGG
  1021 AAGCCCTACACCAAGGAAGCGGCCATGGCCAAGCTGTCTGCCTCCGAAGCGGCCACTTCC
  1081 ATTTCTCACCAGGCGATCCAGATCCTGGGTGGGATGGGCTACGTGACCGAGATGCCCGCA
  1141 GAGCGCCACTACCGGGACGCCCGGATCACCGAGATCTACGAAGGAACCAGCGAGATCCAG
  1201 AGGTTGGTCATTGCAGGGCAGCTGCTGAAGGCGTATCGAGGTTGA

Predicted Protein Product
IGKAYWSMVEPPSREALKQRQAGVRRLHTVYQAVDLPETHLMLRQTCRDFAEKELAPIAA
QLDREHRFPAEQVKKMGSLGLLAMDVPETYGGAGLDYLAYSIAVEEISRGCASTGVIMSV
NNTLYLGPVLKYGSEEQKHKWITPFTNGDKIGCFALSEPGNGSDAGAASTVARLVGDEWV
LNGTKAWITNAWDAAATVVFATTDKGLKHKGISAFLVPMPTPGLSLGKKEDKMGIRASST
ANLIFEDCRIPKDNLLGEEGMGFKIAMQTLDSGRIGIASQALGIAQAALDCAVDYAEKRM
AFGAPITKFQAIQFKLADMALALESARLLTWRAAMLKDNGKPYTKEAAMAKLSASEAATS
ISHQAIQILGGMGYVTEMPAERHYRDARITEIYEGTSEIQRLVIAGQLLKAYRG
Protein Alignment to Mouse
tr|Q91W85|Q91W85_MOUSE Acyl-Coenzyme A dehydrogenase, short chain OS=Mus
            musculus GN=Acads PE=2 SV=1
      MGI:1919235 Acad10 acyl-Coenzyme A dehydrogenase family, member 10 (Chr 5)
        Length = 412

 Score = 1613 (572.9 bits), Expect = 1.7e-166, P = 1.7e-166
 Identities = 318/402 (79%), Positives = 339/402 (84%)

Query:    12 PSREALKQRQAGVRRLHTVYQAVDLPETHLMLRQTCRDFAEKELAPIAAQLDREHRFPAE 71
             P R AL  R    RRLHTVYQ+V+LPETH MLRQTCRDFAEKEL PIAAQLDREH FP  
Sbjct:    12 PLRRALGVRDW--RRLHTVYQSVELPETHQMLRQTCRDFAEKELVPIAAQLDREHLFPTA 69

Query:    72 QVKKMGSLGLLAMDVPETYGGAGLDYLAYSIAVEEISRGCASTGVIMSVNNTLYLGPVLK 131
             QVKKMG LGLLAMDVPE   GAGLDYLAYSIA+EEISR CASTGVIMSVNN+LYLGP+LK
Sbjct:    70 QVKKMGELGLLAMDVPEELSGAGLDYLAYSIALEEISRACASTGVIMSVNNSLYLGPILK 129

Query:   132 YGSEEQKHKWITPFTNGDKIGCFALSEPGNGSDAGAASTVARLVGDEWVLNGTKAWITNX 191
             +GS +QK +WITPFTNGDKIGCFALSEPGNGSDAGAAST AR  GD WVLNGTKAWITN 
Sbjct:   130 FGSAQQKQQWITPFTNGDKIGCFALSEPGNGSDAGAASTTAREEGDSWVLNGTKAWITNS 189

Query:   192 XXXXXXXXXXXXXKGLKHKGISAFLVPMPTPGLSLGKKEDKMGIRASSTANLIFEDCRIP 251
                          +  ++KGISAFLVPMPTPGL+LGKKEDK+GIRASSTANLIFEDCRIP
Sbjct:   190 WEASATVVFASTDRSRQNKGISAFLVPMPTPGLTLGKKEDKLGIRASSTANLIFEDCRIP 249

Query:   252 KDNLLGEEGMGFKIAMQTLDSGRIGIASQALGIAQAALDCAVDYAEKRMAFGAPITKFQA 311
             K+NLLGE GMGFKIAMQTLD GRIGIASQALGIAQA+LDCAV YAE R AFGAP+TK Q 
Sbjct:   250 KENLLGEPGMGFKIAMQTLDMGRIGIASQALGIAQASLDCAVKYAENRNAFGAPLTKLQN 309

Query:   312 IQFKLADMALALESARLLTWRAAMLKDNGKPYTXXXXXXXXXXXXXXTSISHQAIQILGG 371
             IQFKLADMALALESARLLTWRAAMLKDN KP+T              T+ISHQAIQILGG
Sbjct:   310 IQFKLADMALALESARLLTWRAAMLKDNKKPFTKESAMAKLAASEAATAISHQAIQILGG 369

Query:   372 MGYVTEMPAERHYRDARITEIYEGTSEIQRLVIAGQLLKAYR 413
             MGYVTEMPAER+YRDARITEIYEGTSEIQRLVIAG LL++YR
Sbjct:   370 MGYVTEMPAERYYRDARITEIYEGTSEIQRLVIAGHLLRSYR 411