View source for Biology:PCSK5

{{Short description|Protein-coding gene in the species Homo sapiens}}
{{Infobox_gene}}
'''Proprotein convertase subtilisin/kexin type 5''' is an [[Biology:Enzyme|enzyme]] that in humans is encoded by the ''PCSK5'' [[Biology:Gene|gene]], found in chromosome 9q21.3<ref name=Loo>{{cite journal | vauthors = van de Loo JW, Creemers JW, Kas K, Roebroek AJ, Van de Ven WJ | title = Assignment of the human proprotein convertase gene PCSK5 to chromosome 9q21.3 | journal = Cytogenetic and Genome Research | volume = 75 | issue = 4 | pages = 227–229 | year = 1996 | pmid = 9067430 | doi = 10.1159/000134489 }}</ref><ref name="pmid7782070">{{cite journal | vauthors = Mbikay M, Seidah NG, Chrétien M, Simpson EM | title = Chromosomal assignment of the genes for proprotein convertases PC4, PC5, and PACE 4 in mouse and human | journal = Genomics | volume = 26 | issue = 1 | pages = 123–9 | date = Jul 1995 | pmid = 7782070 | doi = 10.1016/0888-7543(95)80090-9 }}</ref><ref name="entrez">{{cite web | title = Entrez Gene: PCSK5 proprotein convertase subtilisin/kexin type 5| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=5125}}</ref>  Two alternatively spliced transcripts are described for this gene but only one has its full length nature known.

== Function ==

The protein encoded by this gene belongs to the [[Biology:Subtilisin|subtilisin]]-like [[Biology:Proprotein convertase|proprotein convertase]] family. The members of this family are proprotein convertases that process latent precursor proteins into their biologically active products. This encoded protein mediates posttranslational endoproteolytic processing for several [[Biology:Integrin|integrin]] alpha subunits. It is thought to process [[Biology:Prorenin|prorenin]], pro-membrane type-1 [[Biology:Matrix metalloproteinase|matrix metalloproteinase]] and HIV-1 glycoprotein gp160.<ref name="entrez" />

== Clinical significance ==

Mutations in this gene have been associated with [[Medicine:Currarino syndrome|Currarino syndrome]]-like malformations.<ref name="pmid18519639">{{cite journal | vauthors = Szumska D, Pieles G, Essalmani R, Bilski M, Mesnard D, Kaur K, Franklyn A, El Omari K, Jefferis J, Bentham J, Taylor JM, Schneider JE, Arnold SJ, Johnson P, Tymowska-Lalanne Z, Stammers D, Clarke K, Neubauer S, Morris A, Brown SD, Shaw-Smith C, Cama A, Capra V, Ragoussis J, Constam D, Seidah NG, Prat A, Bhattacharya S | title = VACTERL/caudal regression/Currarino syndrome-like malformations in mice with mutation in the proprotein convertase Pcsk5 | journal = Genes Dev. | volume = 22 | issue = 11 | pages = 1465–77 | date = June 2008 | pmid = 18519639 | pmc = 2418583 | doi = 10.1101/gad.479408 }}</ref>

== References ==
{{reflist}}

== Further reading ==
{{refbegin | 2}}
* {{cite journal | vauthors = Bassi DE, Mahloogi H, Klein-Szanto AJ | title = The proprotein convertases furin and PACE4 play a significant role in tumor progression | journal = Mol. Carcinog. | volume = 28 | issue = 2 | pages = 63–9 | year = 2000 | pmid = 10900462 | doi = 10.1002/1098-2744(200006)28:2<63::AID-MC1>3.0.CO;2-C | s2cid = 22849623 }}
* {{cite journal | vauthors = Moulard M, Decroly E | title = Maturation of HIV envelope glycoprotein precursors by cellular endoproteases | journal = Biochim. Biophys. Acta | volume = 1469 | issue = 3 | pages = 121–32 | year = 2001 | pmid = 11063880 | doi = 10.1016/S0304-4157(00)00014-9 }}
* {{cite journal | vauthors = Maruyama K, Sugano S | title = Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides | journal = Gene | volume = 138 | issue = 1–2 | pages = 171–4 | year = 1994 | pmid = 8125298 | doi = 10.1016/0378-1119(94)90802-8 }}
* {{cite journal | vauthors = Campan M, Yoshizumi M, Seidah NG, Lee ME, Bianchi C, Haber E | title = Increased proteolytic processing of protein tyrosine phosphatase mu in confluent vascular endothelial cells: the role of PC5, a member of the subtilisin family | journal = Biochemistry | volume = 35 | issue = 12 | pages = 3797–802 | year = 1996 | pmid = 8620001 | doi = 10.1021/bi952552d }}
* {{cite journal | vauthors = Vollenweider F, Benjannet S, Decroly E, Savaria D, Lazure C, Thomas G, Chrétien M, Seidah NG | title = Comparative cellular processing of the human immunodeficiency virus (HIV-1) envelope glycoprotein gp160 by the mammalian subtilisin/kexin-like convertases | journal = Biochem. J. | volume = 314 | issue = Pt 2 | pages = 521–32 | year = 1996 | pmid = 8670066 | pmc = 1217081 | doi =  10.1042/bj3140521}}
* {{cite journal | vauthors = Miranda L, Wolf J, Pichuantes S, Duke R, Franzusoff A | title = Isolation of the human PC6 gene encoding the putative host protease for HIV-1 gp160 processing in CD4+ T lymphocytes | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 93 | issue = 15 | pages = 7695–700 | year = 1996 | pmid = 8755538 | pmc = 38809 | doi = 10.1073/pnas.93.15.7695 | bibcode = 1996PNAS...93.7695M | doi-access = free }}
* {{cite journal | vauthors = Nachtigal MW, Ingraham HA | title = Bioactivation of Müllerian inhibiting substance during gonadal development by a kex2/subtilisin-like endoprotease | journal = Proc. Natl. Acad. Sci. U.S.A. | volume = 93 | issue = 15 | pages = 7711–6 | year = 1996 | pmid = 8755541 | pmc = 38812 | doi = 10.1073/pnas.93.15.7711 | bibcode = 1996PNAS...93.7711N | doi-access = free }}
* {{cite journal | vauthors = Mercure C, Jutras I, Day R, Seidah NG, Reudelhuber TL | title = Prohormone convertase PC5 is a candidate processing enzyme for prorenin in the human adrenal cortex | journal = Hypertension | volume = 28 | issue = 5 | pages = 840–6 | year = 1996 | pmid = 8901832 | doi = 10.1161/01.hyp.28.5.840 }}
* {{cite journal | vauthors = De Bie I, Marcinkiewicz M, Malide D, Lazure C, Nakayama K, Bendayan M, Seidah NG | title = The isoforms of proprotein convertase PC5 are sorted to different subcellular compartments | journal = J. Cell Biol. | volume = 135 | issue = 5 | pages = 1261–75 | year = 1997 | pmid = 8947550 | pmc = 2121096 | doi = 10.1083/jcb.135.5.1261 }}
* {{cite journal | vauthors = van de Loo JW, Creemers JW, Kas K, Roebroek AJ, Van de Ven WJ | title = Assignment of the human proprotein convertase gene PCSK5 to chromosome 9q21.3 | journal = Cytogenet. Cell Genet. | volume = 75 | issue = 4 | pages = 227–9 | year = 1997 | pmid = 9067430 | doi = 10.1159/000134489 }}
* {{cite journal | vauthors = Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S | title = Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library | journal = Gene | volume = 200 | issue = 1–2 | pages = 149–56 | year = 1997 | pmid = 9373149 | doi = 10.1016/S0378-1119(97)00411-3 }}
* {{cite journal | vauthors = Moulard M, Chaloin L, Canarelli S, Mabrouk K, Darbon H, Challoin L | title = Retroviral envelope glycoprotein processing: structural investigation of the cleavage site | journal = Biochemistry | volume = 37 | issue = 13 | pages = 4510–7 | year = 1998 | pmid = 9521771 | doi = 10.1021/bi972662f }}
* {{cite journal | vauthors = Lissitzky JC, Luis J, Munzer JS, Benjannet S, Parat F, Chrétien M, Marvaldi J, Seidah NG | title = Endoproteolytic processing of integrin pro-alpha subunits involves the redundant function of furin and proprotein convertase (PC) 5A, but not paired basic amino acid converting enzyme (PACE) 4, PC5B or PC7 | journal = Biochem. J. | volume = 346 Pt 1 | issue = Pt 1 | pages = 133–8 | year = 2000 | pmid = 10657249 | pmc = 1220832 | doi = 10.1042/0264-6021:3460133 }}
* {{cite journal | vauthors = Yana I, Weiss SJ | title = Regulation of Membrane Type-1 Matrix Metalloproteinase Activation by Proprotein Convertases | journal = Mol. Biol. Cell | volume = 11 | issue = 7 | pages = 2387–401 | year = 2000 | pmid = 10888676 | pmc = 14927 | doi = 10.1091/mbc.11.7.2387 }}
* {{cite journal | vauthors = Hartley JL, Temple GF, Brasch MA | title = DNA Cloning Using In Vitro Site-Specific Recombination | journal = Genome Res. | volume = 10 | issue = 11 | pages = 1788–95 | year = 2001 | pmid = 11076863 | pmc = 310948 | doi = 10.1101/gr.143000 }}
* {{cite journal | vauthors = Simpson JC, Wellenreuther R, Poustka A, Pepperkok R, Wiemann S | title = Systematic subcellular localization of novel proteins identified by large-scale cDNA sequencing | journal = EMBO Rep. | volume = 1 | issue = 3 | pages = 287–92 | year = 2001 | pmid = 11256614 | pmc = 1083732 | doi = 10.1093/embo-reports/kvd058 }}
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{{Sourceattribution|PCSK5}}