CLPP è un gene codificato dal simbolo CLPP. Altri nomi includono: ATP-dependent Clp protease proteolytic subunit, mitochondrial; Endopeptidase Clp. CLPP ha una massa di 30.18kDa, una lunghezza di amminoacidi di 277, ed è implicato in Perrault syndrome 3.
Offriamo 9 anticorpi contro CLPP, allevati nel Coniglio, Topo e Capra, che sono adatti per WB, IHC, ELISA, ICC/IF e Citometria a Flusso con campioni derivati da Umano, Topo, Ratto, Scimmia e Cane.
Informazioni su geni e proteine
Riepilogo UniProt
Protease component of the Clp complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The Clp complex can degrade CSN1S1, CSN2 and CSN3, as well as synthetic peptides (in vitro) and may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates (PubMed:11923310, PubMed:15522782). Cleaves PINK1 in the mitochondrion (PubMed:22354088).
Sommario di Entrez
The protein encoded by this gene belongs to the peptidase family S14 and hydrolyzes proteins into small peptides in the presence of ATP and magnesium. The protein is transported into mitochondrial matrix and is associated with the inner mitochondrial membrane.
Specificità del tessuto
Detected in liver (at protein level). Predominantly expressed in skeletal muscle. Intermediate levels in heart, liver and pancreas. Low in brain, placenta, lung and kidney.
Coinvolgimento nella malattia
Perrault syndrome 3: A sex-influenced disorder characterized by sensorineural deafness in both males and females, and ovarian dysgenesis in females. Affected females have primary amenorrhea, streak gonads, and infertility, whereas affected males show normal pubertal development and are fertile. A spectrum of additional clinical features, including cerebellar ataxia, learning disability, and peripheral neuropathy, have been described in some PRLTS3 affected individuals.
Somiglianze di sequenza
Belongs to the peptidase S14 family.
Posizione cellulare
Mitochondrion matrix.
