KEGG   Homo sapiens (human): 5095
Entry
5095              CDS       T01001                                 

Gene name
PCCA
Definition
(RefSeq) propionyl-CoA carboxylase subunit alpha
  KO
K01965  propionyl-CoA carboxylase alpha chain [EC:6.4.1.3]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00280  Valine, leucine and isoleucine degradation
hsa00630  Glyoxylate and dicarboxylate metabolism
hsa00640  Propanoate metabolism
hsa01100  Metabolic pathways
hsa01200  Carbon metabolism
Module
hsa_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA
Network
nt06024  Valine, leucine and isoleucine degradation
  Element
N00842  Propanoyl-CoA metabolism
N00843  MMUT deficiency in propanoyl-CoA metabolism
N00844  MCEE deficiency in propanoyl-CoA metabolism
N00845  PCCA deficiency in propanoyl-CoA metabolism
Disease
H00175  Propionic acidemia
H01400  Secondary hyperammonemia
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00630 Glyoxylate and dicarboxylate metabolism
    5095 (PCCA)
   00640 Propanoate metabolism
    5095 (PCCA)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    5095 (PCCA)
Enzymes [BR:hsa01000]
 6. Ligases
  6.4  Forming carbon-carbon bonds
   6.4.1  Ligases that form carbon-carbon bonds (only sub-subclass identified to date)
    6.4.1.3  propionyl-CoA carboxylase
     5095 (PCCA)
SSDB
Motif
Pfam: CPSase_L_D2 Biotin_carb_N Biotin_carb_C PCC_BT Biotin_lipoyl Dala_Dala_lig_C Biotin_lipoyl_2 ATP-grasp RimK ATP-grasp_3 HlyD_3 ATP-grasp_5 HlyD_D23 Colicin_immun ATPgrasp_ST ATPgrasp_Ter SLT_beta GARS_A RnfC_N
Other DBs
NCBI-GeneID: 5095
NCBI-ProteinID: NP_000273
OMIM: 232000
HGNC: 8653
Ensembl: ENSG00000175198
Vega: OTTHUMG00000017284
Pharos: P05165(Tbio)
UniProt: P05165
LinkDB
Structure
PDB: 
2JKU 2CQY

Position
13q32.3
AA seq 728 aa
MAGFWVGTAPLVAAGRRGRWPPQQLMLSAALRTLKHVLYYSRQCLMVSRNLGSVGYDPNE
KTFDKILVANRGEIACRVIRTCKKMGIKTVAIHSDVDASSVHVKMADEAVCVGPAPTSKS
YLNMDAIMEAIKKTRAQAVHPGYGFLSENKEFARCLAAEDVVFIGPDTHAIQAMGDKIES
KLLAKKAEVNTIPGFDGVVKDAEEAVRIAREIGYPVMIKASAGGGGKGMRIAWDDEETRD
GFRLSSQEAASSFGDDRLLIEKFIDNPRHIEIQVLGDKHGNALWLNERECSIQRRNQKVV
EEAPSIFLDAETRRAMGEQAVALARAVKYSSAGTVEFLVDSKKNFYFLEMNTRLQVEHPV
TECITGLDLVQEMIRVAKGYPLRHKQADIRINGWAVECRVYAEDPYKSFGLPSIGRLSQY
QEPLHLPGVRVDSGIQPGSDISIYYDPMISKLITYGSDRTEALKRMADALDNYVIRGVTH
NIALLREVIINSRFVKGDISTKFLSDVYPDGFKGHMLTKSEKNQLLAIASSLFVAFQLRA
QHFQENSRMPVIKPDIANWELSVKLHDKVHTVVASNNGSVFSVEVDGSKLNVTSTWNLAS
PLLSVSVDGTQRTVQCLSREAGGNMSIQFLGTVYKVNILTRLAAELNKFMLEKVTEDTSS
VLRSPMPGVVVAVSVKPGDAVAEGQEICVIEAMKMQNSMTAGKTGTVKSVHCQAGDTVGE
GDLLVELE
NT seq 2187 nt   +upstreamnt  +downstreamnt
atggcggggttctgggtcgggacagcaccgctggtcgctgccggacggcgtgggcggtgg
ccgccgcagcagctgatgctgagcgcggcgctgcggaccctgaagcatgttctgtactat
tcaagacagtgcttaatggtgtcccgtaatcttggttcagtgggatatgatcctaatgaa
aaaacttttgataaaattcttgttgctaatagaggagaaattgcatgtcgggttattaga
acttgcaagaagatgggcattaagacagttgccatccacagtgatgttgatgctagttct
gttcatgtgaaaatggcggatgaggctgtctgtgttggcccagctcccaccagtaaaagc
tacctcaacatggatgccatcatggaagccattaagaaaaccagggcccaagctgtacat
ccaggttatggattcctttcagaaaacaaagaatttgccagatgtttggcagcagaagat
gtcgttttcattggacctgacacacatgctattcaagccatgggcgacaagattgaaagc
aaattattagctaagaaagcagaggttaatacaatccctggctttgatggagtagtcaag
gatgcagaagaagctgtcagaattgcaagggaaattggctaccctgtcatgatcaaggcc
tcagcaggtggtggtgggaaaggcatgcgcattgcttgggatgatgaagagaccagggat
ggttttagattgtcatctcaagaagctgcttctagttttggcgatgatagactactaata
gaaaaatttattgataatcctcgtcatatagaaatccaggttctaggtgataaacatggg
aatgctttatggcttaatgaaagagagtgctcaattcagagaagaaatcagaaggtggtg
gaggaagcaccaagcatttttttggatgcggagactcgaagagcgatgggagaacaagct
gtagctcttgccagagcagtaaaatattcctctgctgggaccgtggagttccttgtggac
tctaagaagaatttttatttcttggaaatgaatacaagactccaggttgagcatcctgtc
acagaatgcattactggcctggacctagtccaggaaatgatccgtgttgctaagggctac
cctctcaggcacaaacaagctgatattcgcatcaacggctgggcagttgaatgtcgggtt
tatgctgaggacccctacaagtcttttggtttaccatctattgggagattgtctcagtac
caagaaccgttacatctacctggtgtccgagtggacagtggcatccaaccaggaagtgat
attagcatttattatgatcctatgatttcaaaactaatcacatatggctctgatagaact
gaggcactgaagagaatggcagatgcactggataactatgttattcgaggtgttacacat
aatattgcattacttcgagaggtgataatcaactcacgctttgtaaaaggagacatcagc
actaaatttctctccgatgtgtatcctgatggcttcaaaggacacatgctaaccaagagt
gagaagaaccagttattggcaatagcatcatcattgtttgtggcattccagttaagagca
caacattttcaagaaaattcaagaatgcctgttattaaaccagacatagccaactgggag
ctctcagtaaaattgcatgataaagttcataccgtagtagcatcaaacaatgggtcagtg
ttctcggtggaagttgatgggtcgaaactaaatgtgaccagcacgtggaacctggcttcg
cccttattgtctgtcagcgttgatggcactcagaggactgtccagtgtctttctcgagaa
gcaggtggaaacatgagcattcagtttcttggtacagtgtacaaggtgaatatcttaacc
agacttgccgcagaattgaacaaatttatgctggaaaaagtgactgaggacacaagcagt
gttctgcgttccccgatgcccggagtggtggtggccgtctctgtcaagcctggagacgcg
gtagcagaaggtcaagaaatttgtgtgattgaagccatgaaaatgcagaatagtatgaca
gctgggaaaactggcacggtgaaatctgtgcactgtcaagctggagacacagttggagaa
ggggatctgctcgtggagctggaatga

KEGG   Homo sapiens (human): 5096
Entry
5096              CDS       T01001                                 

Gene name
PCCB
Definition
(RefSeq) propionyl-CoA carboxylase subunit beta
  KO
K01966  propionyl-CoA carboxylase beta chain [EC:6.4.1.3 2.1.3.15]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00280  Valine, leucine and isoleucine degradation
hsa00630  Glyoxylate and dicarboxylate metabolism
hsa00640  Propanoate metabolism
hsa01100  Metabolic pathways
hsa01200  Carbon metabolism
Module
hsa_M00741  Propanoyl-CoA metabolism, propanoyl-CoA => succinyl-CoA
Network
nt06024  Valine, leucine and isoleucine degradation
  Element
N00842  Propanoyl-CoA metabolism
N00843  MMUT deficiency in propanoyl-CoA metabolism
N00844  MCEE deficiency in propanoyl-CoA metabolism
N00846  PCCB deficiency in propanoyl-CoA metabolism
Disease
H00175  Propionic acidemia
H01400  Secondary hyperammonemia
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00630 Glyoxylate and dicarboxylate metabolism
    5096 (PCCB)
   00640 Propanoate metabolism
    5096 (PCCB)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    5096 (PCCB)
Enzymes [BR:hsa01000]
 2. Transferases
  2.1  Transferring one-carbon groups
   2.1.3  Carboxy- and carbamoyltransferases
    2.1.3.15  acetyl-CoA carboxytransferase
     5096 (PCCB)
 6. Ligases
  6.4  Forming carbon-carbon bonds
   6.4.1  Ligases that form carbon-carbon bonds (only sub-subclass identified to date)
    6.4.1.3  propionyl-CoA carboxylase
     5096 (PCCB)
SSDB
Motif
Pfam: Carboxyl_trans MdcE
Other DBs
NCBI-GeneID: 5096
NCBI-ProteinID: NP_000523
OMIM: 232050
HGNC: 8654
Ensembl: ENSG00000114054
Vega: OTTHUMG00000159792
Pharos: P05166(Tbio)
UniProt: P05166
LinkDB
Position
3q22.3
AA seq 539 aa
MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHK
RGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVY
VFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIF
LRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVT
QEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPLSSQDPAPVRECHDPSDRLVP
ELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPK
VASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFA
EATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVE
AAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
NT seq 1620 nt   +upstreamnt  +downstreamnt
atggcggcggcattacgggtggcggcggtcggggcaaggctcagcgttctggcgagcggt
ctccgcgccgcggtccgcagcctttgcagccaggccacctctgttaacgaacgcatcgaa
aacaagcgccggaccgcgctgctgggagggggccaacgccgtattgacgcgcagcacaag
cgaggaaagctaacagccagggagaggatcagtctcttgctggaccctggcagctttgtt
gagagcgacatgtttgtggaacacagatgtgcagattttggaatggctgctgataagaat
aagtttcctggagacagcgtggtcactggacgaggccgaatcaatggaagattggtttat
gtcttcagtcaggattttacagtttttggaggcagtctgtcaggagcacatgcccaaaag
atctgcaaaatcatggaccaggccataacggtgggggctccagtgattgggctgaatgac
tctgggggagcacggatccaagaaggagtggagtctttggctggctatgcagacatcttt
ctgaggaatgttacggcatccggagtcatccctcagatttctctgatcatgggcccatgt
gctggtggggccgtctactccccagccctaacagacttcacgttcatggtaaaggacacc
tcctacctgttcatcactggccctgatgttgtgaagtctgtcaccaatgaggatgttacc
caggaggagctcggtggtgccaagacccacaccaccatgtcaggtgtggcccacagagct
tttgaaaatgatgttgatgccttgtgtaatctccgggatttcttcaactacctgcccctg
agcagtcaggacccggctcccgtccgtgagtgccacgatcccagtgaccgtctggttcct
gagcttgacacaattgtccctttggaatcaaccaaagcctacaacatggtggacatcata
cactctgttgttgatgagcgtgaattttttgagatcatgcccaattatgccaagaacatc
attgttggttttgcaagaatgaatgggaggactgttggaattgttggcaaccaacctaag
gtggcctcaggatgcttggatattaattcatctgtgaaaggggctcgttttgtcagattc
tgtgatgcattcaatattccactcatcacttttgttgatgtccctggctttctacctggc
acagcacaggaatacgggggcatcatccggcatggtgccaagcttctctacgcatttgct
gaggcaactgtacccaaagtcacagtcatcaccaggaaggcctatggaggtgcctatgat
gtcatgagctctaagcacctttgtggtgataccaactatgcctggcccaccgcagagatt
gcagtcatgggagcaaagggcgctgtggagatcatcttcaaagggcatgagaatgtggaa
gctgctcaggcagagtacatcgagaagtttgccaaccctttccctgcagcagtgcgaggg
tttgtggatgacatcatccaaccttcttccacacgtgcccgaatctgctgtgacctggat
gtcttggccagcaagaaggtacaacgtccttggagaaaacatgcaaatattccattgtaa

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