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Sequence of PCCB_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
P05166
Homo sapiens
539
58216
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 MAAALRVAAV GARLSVLASG LRAAVRSLCS QATSVNERIE NKRRTALLGG GQRRIDAQHK
 60 RGKLTARERI SLLLDPGSFV ESDMFVEHRC ADFGMAADKN KFPGDSVVTG RGRINGRLVY
120 VFSQDFTVFG GSLSGAHAQK ICKIMDQAIT VGAPVIGLND SGGARIQEGV ESLAGYADIF
180 LRNVTASGVI PQISLIMGPC AGGAVYSPAL TDFTFMVKDT SYLFITGPDV VKSVTNEDVT
240 QEELGGAKTH TTMSGVAHRA FENDVDALCN LRDFFNYLPL SSQDPAPVRE CHDPSDRLVP
300 ELDTIVPLES TKAYNMVDII HSVVDEREFF EIMPNYAKNI IVGFARMNGR TVGIVGNQPK
360 VASGCLDINS SVKGARFVRF CDAFNIPLIT FVDVPGFLPG TAQEYGGIIR HGAKLLYAFA
420 EATVPKVTVI TRKAYGGAYD VMSSKHLCGD TNYAWPTAEI AVMGAKGAVE IIFKGHENVE
480 AAQAEYIEKF ANPFPAAVRG FVDDIIQPSS TRARICCDLD VLASKKVQRP WRKHANIPL
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Sequence related references
Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
70623
Lamhonwah A.-M.,Leclerc D.,Loyer M.,Clarizio R.,Gravel R.A.
Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit.
Genomics
19
500-505
1994
70624
Ohura T.,Ogasawara M.,Ikeda H.,Narisawa K.,Tada K.
The molecular defect in propionic acidemia: exon skipping caused by an 8-bp deletion from an intron in the PCCB allele.
Hum. Genet.
92
397-402
1993
70625
Rodriguez-Pombo P.,Hoenicka J.,Muro S.,Perez B.,Perez-Cerda C.,Richard E.,Desviat L.R.,Ugarte M.
Human propionyl-CoA carboxylase beta subunit gene: exon-intron definition and mutation spectrum in Spanish and Latin American propionic acidemia patients.
Am. J. Hum. Genet.
63
360-369
1998
70626
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
70627
Muzny D.M.,Scherer S.E.,Kaul R.,Wang J.,Yu J.,Sudbrak R.,Buhay C.J.,Chen R.,Cree A.,Ding Y.,Dugan-Rocha S.,Gill R.,Gunaratne P.,Harris R.A.,Hawes A.C.,Hernandez J.,Hodgson A.V.,Hume J.,Jackson A.,Khan Z.M.,Kovar-Smith C.,Lewis L.R.,Lozado R.J.,Metzker M.L.,Milosavljevic A.,Miner G.R.,Morgan M.B.,Nazareth L.V.,Scott G.,Sodergren E.,Song X.-Z.,Steffen D.,Wei S.,Wheeler D.A.,Wright M.W.,Worley K.C.,Yuan Y.,Zhang Z.,Adams C.Q.,Ansari-Lari M.A.,Ayele M.,Brown M.J.,Chen G.,Chen Z.,Clendenning J.,Clerc-Blankenburg K.P.,Chen R.,Chen Z.,Davis C.,Delgado O.,Dinh H.H.,Dong W.,Draper H.,Ernst S.,Fu G.,Gonzalez-Garay M.L.,Garcia D.K.,Gillett W.,Gu J.,Hao B.,Haugen E.,Havlak P.,He X.,Hennig S.,Hu S.,Huang W.,Jackson L.R.,Jacob L.S.,Kelly S.H.,Kube M.,Levy R.,Li Z.,Liu B.,Liu J.,Liu W.,Lu J.,Maheshwari M.,Nguyen B.-V.,Okwuonu G.O.,Palmeiri A.,Pasternak S.,Perez L.M.,Phelps K.A.,Plopper F.J.,Qiang B.,Raymond C.,Rodriguez R.,Saenphimmachak C.,Santibanez J.,Shen H.,Shen Y.,Subramanian S.,Tabor P.E.,Verduzco D.,Waldron L.,Wang J.,Wang J.,Wang Q.,Williams G.A.,Wong G.K.-S.,Yao Z.,Zhang J.,Zhang X.,Zhao G.,Zhou J.,Zhou Y.,Nelson D.,Lehrach H.,Reinhardt R.,Naylor S.L.,Yang H.,Olson M.,Weinstock G.,Gibbs R.A.
The DNA sequence, annotation and analysis of human chromosome 3.
Nature
440
1194-1198
2006
70629
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
70630
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
70631
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
70633
Tahara T.,Kraus J.P.,Rosenberg L.E.
An unusual insertion/deletion in the gene encoding the beta-subunit of propionyl-CoA carboxylase is a frequent mutation in Caucasian propionic acidemia.
Proc. Natl. Acad. Sci. U.S.A.
87
1372-1376
1990
70634
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
70635
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
70636
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
70637
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
70638
Tahara T.,Kraus J.P.,Ohura T.,Rosenberg L.E.,Fenton W.A.
Three independent mutations in the same exon of the PCCB gene: differences between Caucasian and Japanese propionic acidaemia.
J. Inherit. Metab. Dis.
16
353-360
1993
70639
Muro S.,Rodriguez-Pombo P.,Perez B.,Perez-Cerda C.,Desviat L.R.,Sperl W.,Skladal D.,Sass J.O.,Ugarte M.
Identification of novel mutations in the PCCB gene in European propionic acidemia patients.
Hum. Mutat.
14
89-90
1999
70640
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
70641
Muro S.,Perez B.,Desviat L.R.,Rodriguez-Pombo P.,Perez-Cerda C.,Clavero S.,Ugarte M.
Effect of PCCB gene mutations on the heteromeric and homomeric assembly of propionyl-CoA carboxylase.
Mol. Genet. Metab.
74
476-483
2001
70642
Yorifuji T.,Kawai M.,Muroi J.,Mamada M.,Kurokawa K.,Shigematsu Y.,Hirano S.,Sakura N.,Yoshida I.,Kuhara T.,Endo F.,Mitsubuchi H.,Nakahata T.
Unexpectedly high prevalence of the mild form of propionic acidemia in Japan: presence of a common mutation and possible clinical implications.
Hum. Genet.
111
161-165
2002
70643
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
70644
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
70645
Jiang H.,Rao K.S.,Yee V.C.,Kraus J.P.
Characterization of four variant forms of human propionyl-CoA carboxylase expressed in Escherichia coli.
J. Biol. Chem.
280
27719-27727
2005