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Antibody detects endogenous levels of total LONP1.
This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia. Alternatively spliced transcript variants have been found for this gene.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: h LonHS; hLON ATP-dependent protease; Lon protease homolog, mitochondrial; lon protease homolog, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03120}; Lon protease-like protein; lon protease-like protein {ECO:0000255|HAMAP-Rule:MF_03120}; LONHs; LONP; LONP {ECO:0000255|HAMAP-Rule:MF_03120}; LONP1; Mitochondrial ATP-dependent protease Lon; mitochondrial ATP-dependent protease Lon {ECO:0000255|HAMAP-Rule:MF_03120}; mitochondrial lon protease-like protein; protease, serine, 15; Serine protease 15; serine protease 15 {ECO:0000255|HAMAP-Rule:MF_03120}
基因别名: 1200017E13Rik; CODASS; hLON; LON; LonHS; LONP; LONP1; PIM1; PRSS15
UniProt ID: (Human) P36776, (Mouse) Q8CGK3, (Rat) Q924S5
Entrez Gene ID: (Human) 9361, (Mouse) 74142, (Rat) 170916