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Gene Model: Ivd

NomenclatureGenomic Location
SymbolIvdChromosomeX
NameIsovaleryl coenzyme A
dehydrogenase		Linkage mapunknown
SpeciesDracomimus familiarisGenome CoordinatesChrX: 39 Mbp

Molecular Function

3-methylbutanoyl-CoA + acceptor = 3-methylbut-2-enoyl-CoA + reduced acceptor.

Molecular Function Terms:

binding
   cofactor binding
      coenzyme binding
         flavin adenine dinucleotide binding

catalytic activity
   oxidoreductase activity
      oxidoreductase activity, acting on the CH-CH group of donors
         acyl-CoA dehydrogenase activity

Human Disease Association

Defects in IVD are the cause of isovaleric acidemia (IVA) [MIM:243500]. IVA is characterized by retarded psychomotor development, a peculiar odor resembling sweaty feet, an aversion to dietary protein, and pernicious vomiting, leading to acidosis and coma. The acute neonatal form leads to massive metabolic acidosis from the first days of life and rapid death.

Predicted Transcript
     1 ATGGCGACGGGGGCGCTGCTGAGGAGAGCGGCCTCCGCTTGGGCCCTTCGGTCGGGTCTG
    61 CGGCAGCTCCAGTCCCAGAGAGGATGCGCCGGAGTCGCCGTCGACGACACAGTGAACGGG
   121 CTGAGCGAGGAGCAGCAGCAGCTTAGGCGAACCATGGTGAAATTCTGTCAAGAGCATTTG
   181 GCTCCAAAGGCTCAAGAAATTGATCAGAAGAATGAATTCAAAGAGATGAAGGAGTTTTGG
   241 AAGAAGCTGGGAGAATTAGGAGTTCTGGGAATAACTGCTCCAGCTGAATATGGTGGATCT
   301 GCAATGGGATATCTGGACCATATACTGGTGATGGAAGAAATTTCTCGTGTTTCAGCAGCT
   361 GTTGGATTAAGTTATGGTGCCCACTCTAATCTCTGTGTTAATCAGTTAGTGAGGAATGGA
   421 AATGAAGCACAGAAAGAGAAATACCTGCCTAAGCTAATCAGTGGAGAGCATATTGGAGCA
   481 CTCGCAATGAGTGAAGCCAACGCTGGTTCTGATGTTGTCTCTATGAGGCTGAAGGCAGAC
   541 AGAAAAGGGGATTATTATGTTTTGAATGGGAACAAGTTTTGGATTACCAATGGGCCAGAT
   601 GCTGATGTGCTGATTGTTTATGCTAAAACCGATCCAAGTGCTGTTCCTGCTTCTCAGGGT
   661 ATAACAGCGTTCATTGTGGAAAAGGGTATGCCTGGTTTCAGCACTGCACAAAAGCTTGAT
   721 AAACTGGGGATGAGAGGGTCAAATACCTGTGAATTAATCTTTGAGGACTGCAAGATTCCT
   781 GCTAAGAATGTTTTGGGACAATTGAGTAAAGGTGTCTATGTGTTGATGAGTGGCTTGGAT
   841 CTGGAGAGACTCATATTGTCTGCTGGACCCTTAGGGATCATGCAGGCTGTATTAGACAAT
   901 GCTGTTCCATATATGCATGTGAGAGAAGCTTTTGGGCAGAAAATTGGCCAATTCCAGCTC
   961 ATGCAGGGTAAAATGGCTGACATGTACACTCGGCTGATGTCATGTAGACAGTATGTATAC
  1021 AACGTAGCAAGGGCCTGTGATCGAGGACACTTCAATGCAAAGGATTGCGCTGGAGTGATT
  1081 CTCTACGCTGCTGAATGTGCTACTCAAGTTGCTTTGGATGGAATCCAATGTCTAGGTGGA
  1141 AATGGCTATATCAATGACTACCCTATGGGTCGCTTTTTGCGTGATGCCAAACTGTATGAA
  1201 ATCGGGGCAGGAACCAGTGAAGTACGGAGAATAGTTATTGGCCGCGCATTTAATGCTGCC
  1261 TTTAAATAA

Predicted Protein Product
MATGALLRRAASAWALRSGLRQLQSQRGCAGVAVDDTVNGLSEEQQQLRRTMVKFCQEHL
APKAQEIDQKNEFKEMKEFWKKLGELGVLGITAPAEYGGSAMGYLDHILVMEEISRVSAA
VGLSYGAHSNLCVNQLVRNGNEAQKEKYLPKLISGEHIGALAMSEANAGSDVVSMRLKAD
RKGDYYVLNGNKFWITNGPDADVLIVYAKTDPSAVPASQGITAFIVEKGMPGFSTAQKLD
KLGMRGSNTCELIFEDCKIPAKNVLGQLSKGVYVLMSGLDLERLILSAGPLGIMQAVLDN
AVPYMHVREAFGQKIGQFQLMQGKMADMYTRLMSCRQYVYNVARACDRGHFNAKDCAGVI
LYAAECATQVALDGIQCLGGNGYINDYPMGRFLRDAKLYEIGAGTSEVRRIVIGRAFNAA
FK
Protein Alignment to Mouse
sp|Q9JHI5|IVD_MOUSE Isovaleryl-CoA dehydrogenase, mitochondrial OS=Mus
            musculus GN=Ivd PE=1 SV=1
      MGI:1929242 Ivd isovaleryl coenzyme A dehydrogenase (Chr 2)
        Length = 424

 Score = 1755 (622.8 bits), Expect = 1.6e-181, P = 1.6e-181
 Identities = 328/389 (84%), Positives = 364/389 (93%)

Query:    34 VDDTVNGLSEEQQQLRRTMVKFCQEHLAPKAQEIDQKNEFKEMKEFWKKLGELGVLGITA 93
             VDD +NGL+EEQ+QLR T+ KF QE+LAPKAQEIDQ N+FK ++EFWK+LG LGVLGITA
Sbjct:    36 VDDDINGLNEEQKQLRHTISKFLQENLAPKAQEIDQTNDFKNLREFWKQLGSLGVLGITA 95

Query:    94 PAEYGGSAMGYLDHILVMEEISRVSAAVGLSYGAHSNLCVNQLVRNGNEAQKEKYLPKLI 153
             P +YGGS +GYL+H+LVMEEISR S AVGLSYGAHSNLCVNQ+VRNGNEAQKEKYLPKLI
Sbjct:    96 PVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCVNQIVRNGNEAQKEKYLPKLI 155

Query:   154 SGEHIGALAMSEANAGSDVVSMRLKADRKGDYYVLNGNKFWITNGPDADVLIVYAKTDPS 213
             SGE IGALAMSE NAGSDVVSM+LKA++KGD+YVLNGNKFWITNGPDAD+L+VYAKTD +
Sbjct:   156 SGEFIGALAMSEPNAGSDVVSMKLKAEKKGDHYVLNGNKFWITNGPDADILVVYAKTDLT 215

Query:   214 AVPASQGITAFIVEKGMPGFSTAQKLDKLGMRGSNTCELIFEDCKIPAKNVLGQLSKGVY 273
             AVPAS+GITAFIVEKGMPGFST++KLDKLGMRGSNTCEL+FEDCK+PA NVL Q SKGVY
Sbjct:   216 AVPASRGITAFIVEKGMPGFSTSKKLDKLGMRGSNTCELVFEDCKVPAANVLSQESKGVY 275

Query:   274 VLMSGLDLERLILSAGPLGIMQAVLDNAVPYMHVREAFGQKIGQFQLMQGKMADMYTRLM 333
             VLMSGLDLERL+L+ GPLGIMQAVLD+ +PY+HVREAFGQKIGQFQLMQGKMADMYTRLM
Sbjct:   276 VLMSGLDLERLVLAGGPLGIMQAVLDHTIPYLHVREAFGQKIGQFQLMQGKMADMYTRLM 335

Query:   334 SCRQYVYNVARACDRGHFNAKDCAGVILYAAECATQVALDGIQCLGGNGYINDYPMGRFL 393
             + RQYVYNVA+ACD GH   KDCAGVILYAAECATQVALDGIQCLGGNGYIND+PMGRFL
Sbjct:   336 ASRQYVYNVAKACDEGHIIPKDCAGVILYAAECATQVALDGIQCLGGNGYINDFPMGRFL 395

Query:   394 RDAKLYEIGAGTSEVRRIVIGRAFNAAFK 422
             RDAKLYEIGAGTSEVRR+VIGRAFNA F+
Sbjct:   396 RDAKLYEIGAGTSEVRRLVIGRAFNADFR 424