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Positive test controls include: A-549, HL-60, LO2, HT-1080, BT-474, Mouse heart, Mouse kidney, Mouse liver, Rat kidney. The target is usually found in the following locations: Mitochondrion matrix.
Immunogen sequence: VFPHLPLIAI TRNPVFPRFI KIIEVKNKKL VELLRRKVRL AQPYVGVFLK RDDSNESDVV ESLDEIYHTG TFAQIHEMQD LGDKLRMIVM GHRRVHISRQ LEVEPEEPEA ENKHKPRRKS KRGKKEAEDE LSARHPAELA MEPTPELPAE VLMVEVENVV HEDFQVTEEV KALTAEIVKT IRDIIALNPL YRESVLQMMQ AGQRVVDNPI YLSDMGAALT GAESHELQDV LEETNIPKRL YKALSLLKKE FELSKLQQRL GREVEEKIKQ THRKYLLQEQ L
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.
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Protein Aliases: 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}
Gene Aliases: 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
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