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Gene Model: Mlycd

NomenclatureGenomic Location
SymbolMlycdChromosome1
NameMalonyl-CoA decarboxylaseLinkage mapunknown
SpeciesDracomimus familiarisGenome Coordinates1: 77 Mbp

Molecular Function

Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced

Malonyl-CoA = acetyl-CoA + CO2.

Molecular Function Terms:

catalytic activity
   lyase activity
      carbon-carbon lyase activity
         carboxy-lyase activity

Human Disease Association

Defects in MLYCD are the cause of malonyl-CoA decarboxylase deficiency (MLYCD deficiency) [MIM:248360]. MLYCD deficiency is an autosomal recessive disease characterized by abdominal pain, chronic constipation, episodic vomiting, metabolic acidosis and malonic aciduria.

Predicted Transcript
     1 GACCTGAACAGCACTCTGAAGGCTCTTCTGTCTGAATGGTTCTCTGTTGGGCTCCTCCGA
    61 CTTGAAAGAATCACTTGGCAGTCCCCATGCGAAATCCTTCAGAAGATCAGCCAGTACGAA
   121 GCGGTGCACCCTGTGAGAAACTGGACAGATCTGAAGCGCAGAGTAGGGCCAAACCGGAGG
   181 TGTTACGCCTTTACCCACGCATCCATGCCCGGAGAACCACTGGTGGTGCTGCATGTTGCT
   241 TTAACTGAGGACATAGCAAATAATATCCAGGGCATTGTAAGAGAGTTTGCCACTCTTGAT
   301 GCAGATGAGGATGTGAATAAAATCAACGCCGCCATCTTCTACTCCATCTCTTCCACCCAG
   361 GCCGGACTGCAGGGGGTGGAGCTGGGCAATTACCTGATCAAGAGGGTGGTCCGAGAACTA
   421 CAGAGCGAGTTTCCACACATGGCCCAGTTCTCCAGTCTGTCTCCGATCCCTGGCTTCTCC
   481 TTATGGCTCCAAGGGGCTTTGGGACAGCACAAGAAGGAGGGTCGAGCCACAGAGCTCCTC
   541 TCTGAGCAGGAGTGGCGGGAAGTGGAGGACGTGACGGGAGCTGCTCCTGGTGCCCCGGCT
   601 CTGGAGGCCCTGCGCAGACTCATCAGCACCAGCGAGTGGATTCACTCTGACCGTCTGGTT
   661 CGTGCTTTGGAGCCTGCGCTCATGCGGCTCTGTGCTTGGTATCTGTTTGGTGAAAAACGA
   721 CGAGGGTACGCTCTCAACCCCGTAGCCAACTTTCACCTTCAGAACGGCGCCACCATGTGG
   781 AGGCTAAACTGGCAAGCCGATACAAGCCCAAGAGGAATCGCCAACTCCTGTGGAATAATG
   841 GTCAATTATCGCTACTTTTTGCAAGAGACTGGCACTAACAGCATCGCTTACCTGCACAAT
   901 AAAGTCATCAAGGCCTCGGAGCAGGTGCTGGGACTGGTGTCACAATTTCAGAGGAACAGC
   961 AAACTCTGAGATGAATATGAAGTTCTTTGTTGGGACAGTAATGCATTGGCACTTAATTTC
  1021 AGTGTTATCGTTTTTTGATAACTGAGTGATTTGAATTTAAAGGACAAACACACAATGAGG
  1081 GTGGAATATGCCTTAAATATCCAATGGGATGTACGACTGAAGCATATCATATTTGATTTT
  1141 TATTCAGAGATGAACAGTTGATAATCTGCCTTTCTGAAAGACAAGCATCCAATAAATCCA
  1201 ATTATAACTGGATAGTTCATTCAAAAATGAAA

Predicted Protein Product
DLNSTLKALLSEWFSVGLLRLERITWQSPCEILQKISQYEAVHPVRNWTDLKRRVGPNRR
CYAFTHASMPGEPLVVLHVALTEDIANNIQGIVREFATLDADEDVNKINAAIFYSISSTQ
AGLQGVELGNYLIKRVVRELQSEFPHMAQFSSLSPIPGFSLWLQGALGQHKKEGRATELL
SEQEWREVEDVTGAAPGAPALEALRRLISTSEWIHSDRLVRALEPALMRLCAWYLFGEKR
RGYALNPVANFHLQNGATMWRLNWQADTSPRGIANSCGIMVNYRYFLQETGTNSIAYLHN
KVIKASEQVLGLVSQFQRNSKL
Protein Alignment to Mouse
p|Q99J39|DCMC_MOUSE Malonyl-CoA decarboxylase, mitochondrial OS=Mus musculus
            GN=Mlycd PE=2 SV=1
      MGI:1928485 Mlycd malonyl-CoA decarboxylase (Chr 8)
        Length = 492

 Score = 1082 (385.9 bits), Expect = 3.2e-110, P = 3.2e-110
 Identities = 207/323 (64%), Positives = 251/323 (77%)

Query:     1 DLNSTLKALLSEWFSVGLLRLERITWQSPCEILQKISQYEAVHPVRNWTDLKRRVGPNRR 60
             ++N  LK++LSEWFS G L LER+TW SPCE+LQKIS+ EAVHPV+NW D+KRRVGP RR
Sbjct:   176 EMNGVLKSMLSEWFSSGFLNLERVTWHSPCEVLQKISECEAVHPVKNWMDMKRRVGPYRR 235

Query:    61 CYAFTHASMPGEPLVVLHVALTEDIANNIQGIVREFATLDADEDVNKINAAIFYSISSTQ 120
             CY F+H S PGEPLVVLHVALT DI+NNIQGIV+E    + +E  N+I AAIFYSIS TQ
Sbjct:   236 CYFFSHCSTPGEPLVVLHVALTGDISNNIQGIVKECPPTETEER-NRIAAAIFYSISLTQ 294

Query:   121 AGLQGVELGNYLIKRVVRELQSEFPHMAQFSSLSPIPGFSLWLQGALG-QHKKEGRATEL 179
              GLQGVELG +LIKRVV+ELQ EFP +  FSSLSPIPGF+ WL G L  Q K+ GR  EL
Sbjct:   295 QGLQGVELGTFLIKRVVKELQKEFPQLGAFSSLSPIPGFTKWLLGLLNVQGKEHGR-NEL 353

Query:   180 LSEQEWREVEDVTXXXXXXXXXXXXXXLISTSEWIHSDRLVRALEPALMRLCAWYLFGEK 239
              ++ E +E+  VT               +S+ EW+ S++L +AL+  LMRLCAWYL+GEK
Sbjct:   354 FTDSECQEISAVTGNPVHESLKG----FLSSGEWVKSEKLTQALQGPLMRLCAWYLYGEK 409

Query:   240 RRGYALNPVANFHLQNGATMWRLNWQADTSPRGIANSCGIMVNYRYFLQETGTNSIAYLH 299
              RGYALNPVANFHLQNGA MWR+NW AD+S +G+ +SCG+MVNYRY+L+ETG NSI+YL 
Sbjct:   410 HRGYALNPVANFHLQNGAVMWRINWMADSSLKGLTSSCGLMVNYRYYLEETGPNSISYLG 469

Query:   300 NKVIKASEQVLGLVSQFQRNSKL 322
             +K IKASEQ+L LV+QFQ NSKL
Sbjct:   470 SKNIKASEQILSLVAQFQNNSKL 492