Sequence of PCCA_HUMAN

EC Number:6.4.1.3

EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
propionyl-CoA carboxylase
P05165
Homo sapiens
728
80059
Reaction
ATP + propanoyl-CoA + HCO3- + H+ = ADP + phosphate + (S)-methylmalonyl-CoA
Other sequences found for EC No. 6.4.1.3

General information:

Sequence
show sequence in fasta format
  0 MAGFWVGTAP LVAAGRRGRW PPQQLMLSAA LRTLKHVLYY SRQCLMVSRN LGSVGYDPNE
 60 KTFDKILVAN RGEIACRVIR TCKKMGIKTV AIHSDVDASS VHVKMADEAV CVGPAPTSKS
120 YLNMDAIMEA IKKTRAQAVH PGYGFLSENK EFARCLAAED VVFIGPDTHA IQAMGDKIES
180 KLLAKKAEVN TIPGFDGVVK DAEEAVRIAR EIGYPVMIKA SAGGGGKGMR IAWDDEETRD
240 GFRLSSQEAA SSFGDDRLLI EKFIDNPRHI EIQVLGDKHG NALWLNEREC SIQRRNQKVV
300 EEAPSIFLDA ETRRAMGEQA VALARAVKYS SAGTVEFLVD SKKNFYFLEM NTRLQVEHPV
360 TECITGLDLV QEMIRVAKGY PLRHKQADIR INGWAVECRV YAEDPYKSFG LPSIGRLSQY
420 QEPLHLPGVR VDSGIQPGSD ISIYYDPMIS KLITYGSDRT EALKRMADAL DNYVIRGVTH
480 NIALLREVII NSRFVKGDIS TKFLSDVYPD GFKGHMLTKS EKNQLLAIAS SLFVAFQLRA
540 QHFQENSRMP VIKPDIANWE LSVKLHDKVH TVVASNNGSV FSVEVDGSKL NVTSTWNLAS
600 PLLSVSVDGT QRTVQCLSRE AGGNMSIQFL GTVYKVNILT RLAAELNKFM LEKVTEDTSS
660 VLRSPMPGVV VAVSVKPGDA VAEGQEICVI EAMKMQNSMT AGKTGTVKSV HCQAGDTVGE
720 GDLLVELE
Download this sequence
in fasta format
Download all sequences for 6.4.1.3
in fasta format
in csv (Excel, OpenOffice) format
Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
70572
Campeau E.,Desviat L.R.,Leclerc D.,Wu X.,Perez B.,Ugarte M.,Gravel R.A.
Structure of the PCCA gene and distribution of mutations causing propionic acidemia.
Mol. Genet. Metab.
74
238-247
2001
70573
Ota T.,Suzuki Y.,Nishikawa T.,Otsuki T.,Sugiyama T.,Irie R.,Wakamatsu A.,Hayashi K.,Sato H.,Nagai K.,Kimura K.,Makita H.,Sekine M.,Obayashi M.,Nishi T.,Shibahara T.,Tanaka T.,Ishii S.,Yamamoto J.,Saito K.,Kawai Y.,Isono Y.,Nakamura Y.,Nagahari K.,Murakami K.,Yasuda T.,Iwayanagi T.,Wagatsuma M.,Shiratori A.,Sudo H.,Hosoiri T.,Kaku Y.,Kodaira H.,Kondo H.,Sugawara M.,Takahashi M.,Kanda K.,Yokoi T.,Furuya T.,Kikkawa E.,Omura Y.,Abe K.,Kamihara K.,Katsuta N.,Sato K.,Tanikawa M.,Yamazaki M.,Ninomiya K.,Ishibashi T.,Yamashita H.,Murakawa K.,Fujimori K.,Tanai H.,Kimata M.,Watanabe M.,Hiraoka S.,Chiba Y.,Ishida S.,Ono Y.,Takiguchi S.,Watanabe S.,Yosida M.,Hotuta T.,Kusano J.,Kanehori K.,Takahashi-Fujii A.,Hara H.,Tanase T.-O.,Nomura Y.,Togiya S.,Komai F.,Hara R.,Takeuchi K.,Arita M.,Imose N.,Musashino K.,Yuuki H.,Oshima A.,Sasaki N.,Aotsuka S.,Yoshikawa Y.,Matsunawa H.,Ichihara T.,Shiohata N.,Sano S.,Moriya S.,Momiyama H.,Satoh N.,Takami S.,Terashima Y.,Suzuki O.,Nakagawa S.,Senoh A.,Mizoguchi H.,Goto Y.,Shimizu F.,Wakebe H.,Hishigaki H.,Watanabe T.,Sugiyama A.,Takemoto M.,Kawakami B.,Yamazaki M.,Watanabe K.,Kumagai A.,Itakura S.,Fukuzumi Y.,Fujimori Y.,Komiyama M.,Tashiro H.,Tanigami A.,Fujiwara T.,Ono T.,Yamada K.,Fujii Y.,Ozaki K.,Hirao M.,Ohmori Y.,Kawabata A.,Hikiji T.,Kobatake N.,Inagaki H.,Ikema Y.,Okamoto S.,Okitani R.,Kawakami T.,Noguchi S.,Itoh T.,Shigeta K.,Senba T.,Matsumura K.,Nakajima Y.,Mizuno T.,Morinaga M.,Sasaki M.,Togashi T.,Oyama M.,Hata H.,Watanabe M.,Komatsu T.,Mizushima-Sugano J.,Satoh T.,Shirai Y.,Takahashi Y.,Nakagawa K.,Okumura K.,Nagase T.,Nomura N.,Kikuchi H.,Masuho Y.,Yamashita R.,Nakai K.,Yada T.,Nakamura Y.,Ohara O.,Isogai T.,Sugano S.
Complete sequencing and characterization of 21,243 full-length human cDNAs.
Nat. Genet.
36
40-45
2004
70574
Dunham A.,Matthews L.H.,Burton J.,Ashurst J.L.,Howe K.L.,Ashcroft K.J.,Beare D.M.,Burford D.C.,Hunt S.E.,Griffiths-Jones S.,Jones M.C.,Keenan S.J.,Oliver K.,Scott C.E.,Ainscough R.,Almeida J.P.,Ambrose K.D.,Andrews D.T.,Ashwell R.I.S.,Babbage A.K.,Bagguley C.L.,Bailey J.,Bannerjee R.,Barlow K.F.,Bates K.,Beasley H.,Bird C.P.,Bray-Allen S.,Brown A.J.,Brown J.Y.,Burrill W.,Carder C.,Carter N.P.,Chapman J.C.,Clamp M.E.,Clark S.Y.,Clarke G.,Clee C.M.,Clegg S.C.,Cobley V.,Collins J.E.,Corby N.,Coville G.J.,Deloukas P.,Dhami P.,Dunham I.,Dunn M.,Earthrowl M.E.,Ellington A.G.,Faulkner L.,Frankish A.G.,Frankland J.,French L.,Garner P.,Garnett J.,Gilbert J.G.R.,Gilson C.J.,Ghori J.,Grafham D.V.,Gribble S.M.,Griffiths C.,Hall R.E.,Hammond S.,Harley J.L.,Hart E.A.,Heath P.D.,Howden P.J.,Huckle E.J.,Hunt P.J.,Hunt A.R.,Johnson C.,Johnson D.,Kay M.,Kimberley A.M.,King A.,Laird G.K.,Langford C.J.,Lawlor S.,Leongamornlert D.A.,Lloyd D.M.,Lloyd C.,Loveland J.E.,Lovell J.,Martin S.,Mashreghi-Mohammadi M.,McLaren S.J.,McMurray A.,Milne S.,Moore M.J.F.,Nickerson T.,Palmer S.A.,Pearce A.V.,Peck A.I.,Pelan S.,Phillimore B.,Porter K.M.,Rice C.M.,Searle S.,Sehra H.K.,Shownkeen R.,Skuce C.D.,Smith M.,Steward C.A.,Sycamore N.,Tester J.,Thomas D.W.,Tracey A.,Tromans A.,Tubby B.,Wall M.,Wallis J.M.,West A.P.,Whitehead S.L.,Willey D.L.,Wilming L.,Wray P.W.,Wright M.W.,Young L.,Coulson A.,Durbin R.M.,Hubbard T.,Sulston J.E.,Beck S.,Bentley D.R.,Rogers J.,Ross M.T.
The DNA sequence and analysis of human chromosome 13.
Nature
428
522-528
2004
70576
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
70577
Lamhonwah A.-M.,Mahuran D.J.,Gravel R.A.
Human mitochondrial propionyl-CoA carboxylase: localization of the N-terminus of the pro- and mature alpha chains in the deduced primary sequence of a full-length cDNA.
Nucleic Acids Res.
17
4396-4396
1989
70579
Stadler S.C.,Polanetz R.,Meier S.,Mayerhofer P.U.,Herrmann J.M.,Anslinger K.,Roscher A.A.,Roschinger W.,Holzinger A.
Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase.
Biochem. Biophys. Res. Commun.
334
939-946
2005
70580
Stankovics J.,Ledley F.D.
Cloning of functional alpha propionyl CoA carboxylase and correction of enzyme deficiency in pccA fibroblasts.
Am. J. Hum. Genet.
52
144-151
1993
70581
Lamhonwah A.-M.,Barankiewicz T.J.,Willard H.F.,Mahuran D.J.,Quan F.,Gravel R.A.
Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes.
Proc. Natl. Acad. Sci. U.S.A.
83
4864-4868
1986
70582
Lamhonwah A.-M.,Quan F.,Gravel R.A.
Sequence homology around the biotin-binding site of human propionyl-CoA carboxylase and pyruvate carboxylase.
Arch. Biochem. Biophys.
254
631-636
1987
70583
Kalousek F.,Darigo M.D.,Rosenberg L.E.
Isolation and characterization of propionyl-CoA carboxylase from normal human liver. Evidence for a protomeric tetramer of nonidentical subunits.
J. Biol. Chem.
255
60-65
1980
70584
Leon-Del-Rio A.,Leclerc D.,Akerman B.,Wakamatsu N.,Gravel R.A.
Isolation of a cDNA encoding human holocarboxylase synthetase by functional complementation of a biotin auxotroph of Escherichia coli.
Proc. Natl. Acad. Sci. U.S.A.
92
4626-4630
1995
70585
Huang C.S.,Sadre-Bazzaz K.,Shen Y.,Deng B.,Zhou Z.H.,Tong L.
Crystal structure of the alpha(6)beta(6) holoenzyme of propionyl-coenzyme A carboxylase.
Nature
466
1001-1005
2010
70586
Burkard T.R.,Planyavsky M.,Kaupe I.,Breitwieser F.P.,Buerckstuemmer T.,Bennett K.L.,Superti-Furga G.,Colinge J.
Initial characterization of the human central proteome.
BMC Syst. Biol.
5
17-17
2011
70587
Wirth M.,Karaca S.,Wenzel D.,Ho L.,Tishkoff D.,Lombard D.B.,Verdin E.,Urlaub H.,Jedrusik-Bode M.,Fischle W.
Mitochondrial SIRT4-type proteins in Caenorhabditis elegans and mammals interact with pyruvate carboxylase and other acetylated biotin-dependent carboxylases.
Mitochondrion
13
705-720
2013
70588
Bian Y.,Song C.,Cheng K.,Dong M.,Wang F.,Huang J.,Sun D.,Wang L.,Ye M.,Zou H.
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
J. Proteomics
96
253-262
2014
70590
Healy S.,McDonald M.K.,Wu X.,Yue W.W.,Kochan G.,Oppermann U.,Gravel R.A.
Structural impact of human and Escherichia coli biotin carboxyl carrier proteins on biotin attachment.
Biochemistry
49
4687-4694
2010
70591
Ugarte M.,Perez-Cerda C.,Rodriguez-Pombo P.,Desviat L.R.,Perez B.,Richard E.,Muro S.,Campeau E.,Ohura T.,Gravel R.A.
Overview of mutations in the PCCA and PCCB genes causing propionic acidemia.
Hum. Mutat.
14
275-282
1999
70592
Richard E.,Desviat L.R.,Perez B.,Perez-Cerda C.,Ugarte M.
Genetic heterogeneity in propionic acidemia patients with alpha-subunit defects: identification of five novel mutations, one of them causing instability of the protein.
Biochim. Biophys. Acta
1453
351-358
1999
70593
Perez B.,Desviat L.R.,Rodriguez-Pombo P.,Clavero S.,Navarrete R.,Perez-Cerda C.,Ugarte M.
Propionic acidemia: identification of twenty-four novel mutations in Europe and North America.
Mol. Genet. Metab.
78
59-67
2003
70594
Yang X.,Sakamoto O.,Matsubara Y.,Kure S.,Suzuki Y.,Aoki Y.,Yamaguchi S.,Takahashi Y.,Nishikubo T.,Kawaguchi C.,Yoshioka A.,Kimura T.,Hayasaka K.,Kohno Y.,Iinuma K.,Ohura T.
Mutation spectrum of the PCCA and PCCB genes in Japanese patients with propionic acidemia.
Mol. Genet. Metab.
81
335-342
2004
70595
Campeau E.,Dupuis L.,Leon-Del-Rio A.,Gravel R.
Coding sequence mutations in the alpha subunit of propionyl-CoA carboxylase in patients with propionic acidemia.
Mol. Genet. Metab.
67
11-22
1999