KEGG T01001: 5096

Homo sapiens (human): 5096

Entry

5096 CDS T01001

Gene name

PCCB

Definition

(RefSeq) propionyl-CoA carboxylase subunit beta

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:

NCBI-ProteinID:

OMIM:

HGNC:

Ensembl:

Vega:

Pharos:

P05166(Tbio)

UniProt:

P05166

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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