Tripeptidyl peptidase
From Proteopedia
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<StructureSection load='3ee6' size='340' side='right' caption='Glycosylated tripeptidyl peptidase I dimer complex with sulfate, Zn+2 (grey), Ca+2 (green) and Cl- (green) ions, [[3ee6]]' scene=''> | <StructureSection load='3ee6' size='340' side='right' caption='Glycosylated tripeptidyl peptidase I dimer complex with sulfate, Zn+2 (grey), Ca+2 (green) and Cl- (green) ions, [[3ee6]]' scene=''> | ||
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== Didease == | == Didease == | ||
| - | TPP-I participates in lysosomal turnover of proteins in pathological conditions associated with cell injury<ref>PMID:11589013</ref>. TPP-II deficiency is linked to severe autoimmunity<ref>PMID:25414442</ref>. TPP-II has central and peripheral roles in metabolism and hence in fat formation<ref>PMID:17932511</ref>. | + | TPP-I participates in lysosomal turnover of proteins in pathological conditions associated with cell injury and its mutations cause the fatal neurodegenerative childhood neuronal ceroid lipofuscinosis<ref>PMID:11589013</ref>. TPP-II deficiency is linked to severe autoimmunity<ref>PMID:25414442</ref>. TPP-II has central and peripheral roles in metabolism and hence in fat formation<ref>PMID:17932511</ref>. |
== Structural highlights == | == Structural highlights == | ||
Revision as of 10:27, 22 September 2016
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3D Structures of Tripeptidyl peptidase
Updated on 22-September-2016
References
- ↑ Wisniewski KE, Kida E, Walus M, Wujek P, Kaczmarski W, Golabek AA. Tripeptidyl-peptidase I in neuronal ceroid lipofuscinoses and other lysosomal storage disorders. Eur J Paediatr Neurol. 2001;5 Suppl A:73-9. PMID:11589013
- ↑ Stepensky P, Rensing-Ehl A, Gather R, Revel-Vilk S, Fischer U, Nabhani S, Beier F, Brummendorf TH, Fuchs S, Zenke S, Firat E, Pessach VM, Borkhardt A, Rakhmanov M, Keller B, Warnatz K, Eibel H, Niedermann G, Elpeleg O, Ehl S. Early-onset Evans syndrome, immunodeficiency, and premature immunosenescence associated with tripeptidyl-peptidase II deficiency. Blood. 2015 Jan 29;125(5):753-61. doi: 10.1182/blood-2014-08-593202. Epub 2014, Nov 20. PMID:25414442 doi:http://dx.doi.org/10.1182/blood-2014-08-593202
- ↑ McKay RM, McKay JP, Suh JM, Avery L, Graff JM. Tripeptidyl peptidase II promotes fat formation in a conserved fashion. EMBO Rep. 2007 Dec;8(12):1183-9. Epub 2007 Oct 12. PMID:17932511 doi:http://dx.doi.org/10.1038/sj.embor.7401086
- ↑ Pal A, Kraetzner R, Gruene T, Grapp M, Schreiber K, Gronborg M, Urlaub H, Becker S, Asif AR, Gartner J, Sheldrick GM, Steinfeld R. Structure of tripeptidyl-peptidase I provides insight into the molecular basis of late infantile neuronal ceroid lipofuscinosis. J Biol Chem. 2009 Feb 6;284(6):3976-84. Epub 2008 Nov 26. PMID:19038966 doi:10.1074/jbc.M806947200
