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Gene Model: Pccb

NomenclatureGenomic Location
SymbolPccbChromosomeX
NamePropionyl-Coenzyme A carboxylase,
beta polypeptide		Linkage mapunknown
SpeciesDracomimus familiarisGenome CoordinatesX: 24 Mbp

Molecular Function

ATP + propanoyl-CoA + HCO3- = ADP + phosphate + (S)-methylmalonyl-CoA.

Molecular Function Terms:

catalytic activity
   ligase activity
      ligase activity, forming carbon-nitrogen bonds
         CoA carboxylase activity

Human Disease Association

Defects in PCCB are the cause of propionic acidemia type II (PA-2) [MIM:606054]. PA-2 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.

Predicted Transcript
     1 CAGACCGTCATGGCGTCTATGCGGCTGCTTCTCGCCTCCTGGCGCCCGGTGTCGAGGCTT
    61 TGCCGGCCCTTCAGTACCAGCTCCTCGCTCCGCGCCCTCACGGTCCCCGAGCGCATCGAG
   121 GAGAAGCGCCGGGCGGCCATGCTGGGCGGGGGAAAGGCCCGCATAGAGGCCCAGCACCGC
   181 CGGGGTAAGCTCACAGCCAGGGAGCGCGTTCTTCTCTTACTGGATCCTGACAGTTTTGTA
   241 GAGTATGACATGTTTGTGGAACACAGATGTTCAGATTTTGGGATGGAGGATGACAAAAAC
   301 AAGTACCCTGGAGACAGTGTGGTAACTGGTCAGGGTAGAATTAATGGTAGGCTGGCATAT
   361 GTCTTCAGTCAGGATTTCACTGTCTTTGGGGGCAGTTTATCTGGTGCGCATGCTCAAAAG
   421 ATCTGCAAGATCATGGATCAGGCAGCAATGGTTGGTGCTCCTGTGATTGGACTGAATGAT
   481 TCAGGAGGAGCCCGCATCCAAGAAGGAGTAGAGTCACTGGCAGGTTATGCTGATATTTTT
   541 TTGAGAAATGTTTTGTGTTCTGGAGTAGTGCCACAAATCTCTCTTATCATGGGCCCCTGT
   601 GCAGGAGGTGCTGTCTATTCTCCTGCGTTGACAGATTTCACATTTATGGTTAAGGATACA
   661 TCTTATCTGTTTATTACAGGCCCTGATGTTGTCAAATCAGTTACCAATGAAGATGTTACT
   721 CAGGAGCAACTTGGTGGTGCAAAAACACACAGTGCAGTATCAGGGGTTGCACACAATGCT
   781 TTTGAGAATGATGTAGATGCTTTGCTCAATCTCCGAGAGTTTTTTAATTACTTACCTCTT
   841 AGTAATAAAGACCCAGCTCCTGTCAGAGAATGCCATGACCCTAGCAACCGCTTGGTACCT
   901 GAGTTGGATACTATTGTCCCAATGGAATCAACCAAAGCTTATGATATGGTGGATATTATA
   961 CATGCGATTGTTGATGAGAGAGAATTCTTTGAGATTATGCCTTCCTATGCCAGAAACATT
  1021 GTAATTGGGTTTGCCAGAATGAATGGAAGAACGGTTGGAATAGTCGGCAATCAGCCCAAA
  1081 GTAGCCTCAGGTTGTTTGGATATAAACTCTTCTGTGAAAGGAGCCCGTTTTGTTCGCTTC
  1141 TGTGATGCTTTCAACATACCTCTGATAACATTTGTAGATGTGCCTGGATTCTTACCAGGG
  1201 ACAGCTCAAGAATACGGTGGGATTATACGTCACGGTGCAAAACTGCTCTTTGCTTTTGCA
  1261 GAAGCCACAGTGCCAAAAATTACTATTATTACAAGGAAGGCTTACGGTGGAGCATATGAT
  1321 GTGATGAGTTCTAAGCATCTGAGAGGAGATGCAAATTATGCCTGGCCAACAGCGGAAGTA
  1381 GCTGTGATGGGAGCCAAGGGAGCTGTTCAGATAATTTTCAGAGGAAAAGGGGCACATGCA
  1441 GAGGCAGAGTATGTGGAAAAGTTTGCAAACCCTTTCCCTGCAGCAACCAGAGGGTTCGTT
  1501 GATGACATTATTCAACCTTCTTCTACTCGACTTCGTATTTGCCGTGATTTAGAAGTTTTA
  1561 GCCAGTAAAAAACAGTCCAACCCCTGGAAGAAGCATGCCAATATTCCACTTTGA

Predicted Protein Product
QTVMASMRLLLASWRPVSRLCRPFSTSSSLRALTVPERIEEKRRAAMLGGGKARIEAQHR
RGKLTARERVLLLLDPDSFVEYDMFVEHRCSDFGMEDDKNKYPGDSVVTGQGRINGRLAY
VFSQDFTVFGGSLSGAHAQKICKIMDQAAMVGAPVIGLNDSGGARIQEGVESLAGYADIF
LRNVLCSGVVPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVT
QEQLGGAKTHSAVSGVAHNAFENDVDALLNLREFFNYLPLSNKDPAPVRECHDPSNRLVP
ELDTIVPMESTKAYDMVDIIHAIVDEREFFEIMPSYARNIVIGFARMNGRTVGIVGNQPK
VASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLFAFA
EATVPKITIITRKAYGGAYDVMSSKHLRGDANYAWPTAEVAVMGAKGAVQIIFRGKGAHA
EAEYVEKFANPFPAATRGFVDDIIQPSSTRLRICRDLEVLASKKQSNPWKKHANIPL
Protein Alignment to Mouse
tr|A0PJE6|A0PJE6_MOUSE Pccb protein (Fragment) OS=Mus musculus GN=Pccb PE=2
            SV=1
      MGI:108451 Acaca acetyl-Coenzyme A carboxylase alpha (Chr 11)
        Length = 539

 Score = 2316 (820.3 bits), Expect = 5.5e-241, P = 5.5e-241
 Identities = 439/507 (86%), Positives = 475/507 (93%)

Query:    33 LTVPERIEEKRRAAMLGGGKARIEAQHRRGKLTARERVLLLLDPDSFVEYDMFVEHRCSD 92
             ++V ERI+ KR AA+LGGG+ RI+AQH+RGKLTARER+ LLLDP SFVE DMFVEHRC+D
Sbjct:    33 VSVKERIDNKRHAALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCAD 92

Query:    93 FGMEDDKNKYPGDSVVTGQGRINGRLAYVFSQDFTVFGGSLSGAHAQKICKIMDQAAMVG 152
             FGM  DKNK+PGDSVVTG+GRINGRL YVFSQDFTVFGGSLSGAHAQKICKIMDQA  VG
Sbjct:    93 FGMAADKNKFPGDSVVTGRGRINGRLVYVFSQDFTVFGGSLSGAHAQKICKIMDQAITVG 152

Query:   153 APVIGLNDSGGARIQEGVESLAGYADIFLRNVLCSGVVPQISLIMGPCAGGAVYSPALTD 212
             APVIGLNDSGGARIQEGVESLAGYADIFLRNV  SGV+PQISLIMGPCAGGAVYSPALTD
Sbjct:   153 APVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPCAGGAVYSPALTD 212

Query:   213 FTFMVKDTSYLFITGPDVVKSVTNEDVTQEQLGGAKTHSAVSGVAHNAFENDVDALLNLR 272
             FTFMVKDTSYLFITGP+VVKSVTNEDVTQEQLGGAKTH+ VSGVAH AF+NDVDAL NLR
Sbjct:   213 FTFMVKDTSYLFITGPEVVKSVTNEDVTQEQLGGAKTHTTVSGVAHRAFDNDVDALCNLR 272

Query:   273 EFFNYLPLSNKDPAPVRECHDPSNRLVPELDTIVPMESTKAYDMVDIIHAIVDEREFFEI 332
             EFFN+LPLS++DPAP+RECHDPS+RLVPELDT+VP+ES+KAY+M+DIIHA++DEREFFEI
Sbjct:   273 EFFNFLPLSSQDPAPIRECHDPSDRLVPELDTVVPLESSKAYNMLDIIHAVIDEREFFEI 332

Query:   333 MPSYARNIVIGFARMNGRTVGIVGNQPKVASGCLDINSSVKGARFVRFCDAFNIPLITFV 392
             MPSYA+NIV+GFARMNGRTVGIVGNQP VASGCLDINSSVKGARFVRFCDAFNIPLITFV
Sbjct:   333 MPSYAKNIVVGFARMNGRTVGIVGNQPNVASGCLDINSSVKGARFVRFCDAFNIPLITFV 392

Query:   393 DVPGFLPGTAQEYGGIIRHGAKLLFAFAEATVPKITIITRKAYGGAYDVMSSKHLRGDAN 452
             DVPGFLPGTAQEYGGIIRHGAKLL+AFAEATVPKIT+ITRKAYGGAYDVMSSKHL GD N
Sbjct:   393 DVPGFLPGTAQEYGGIIRHGAKLLYAFAEATVPKITVITRKAYGGAYDVMSSKHLLGDTN 452

Query:   453 YAWPTAEVAVMGAKGAVQIIFRGKG--AHAEAEYVEKFANPFPAATRGFVDDIIQPSSTR 510
             YAWPTAE+AVMGAKGAV+IIF+G      A+AEYVEKFANPFPAA RGFVDDIIQPSSTR
Sbjct:   453 YAWPTAEIAVMGAKGAVEIIFKGHQDVEAAQAEYVEKFANPFPAAVRGFVDDIIQPSSTR 512

Query:   511 LRICRDLEVLASKKQSNPWKKHANIPL 537
              RIC DLEVLASKK   PW+KHANIPL
Sbjct:   513 ARICCDLEVLASKKVHRPWRKHANIPL 539