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Unless otherwise stated all data on this page refer to the human proteins. Gene information is provided for human (Hs), mouse (Mm) and rat (Rn).


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AAA or AAA+ (ATPases Associated with diverse cellular Activities) proteins couple chemical energy provided by ATP hydrolysis to the remodeling or translocation of macromolecules. They are involved in a wide range of cellular processes, including DNA replication, protein degradation, membrane fusion, microtubule severing, peroxisome biogenesis, signal transduction and the regulation of gene expression.


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katanin catalytic subunit A1 Show summary » More detailed page go icon to follow link

lon peptidase 1, mitochondrial Show summary »

Target Id 3180
Nomenclature lon peptidase 1, mitochondrial
Previous and unofficial names PRSS15
Genes LONP1 (Hs), Lonp1 (Mm), Lonp1 (Rn)
Ensembl ID ENSG00000196365 (Hs), ENSMUSG00000041168 (Mm), ENSRNOG00000046502 (Rn)
UniProtKB AC P36776 (Hs), Q8CGK3 (Mm), Q924S5 (Rn)
EC number
compound 12d [PMID: 33821636] pIC50 7.8 [2]
Comment LONP1 is an AAA+ serine protease. It mediates the selective degradation of misfolded, unassembled or oxidatively damaged proteins in the mitochondrial matrix, and is essential for mitochondrial homeostasis. Dysregulation of LONP1 leads to disease: Loss of function leads to severe developmental complications in CODAS syndrome [1] and other mitochondrial diseases [4], whereas elevated expression exerts pro-tumourigenic effects in some cancers [3,5-6]. Homozygous LONP1 knockouts are embryonically lethal.


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How to cite this family page

Database page citation:

AAA ATPases. Accessed on 22/10/2021. IUPHAR/BPS Guide to PHARMACOLOGY,

Concise Guide to PHARMACOLOGY citation:

Alexander SPH, Fabbro D, Kelly E, Mathie A, Peters JA, Veale EL, Armstrong JF, Faccenda E, Harding SD, Pawson AJ, Sharman JL, Southan C, Davies JA; CGTP Collaborators. (2019) The Concise Guide to PHARMACOLOGY 2019/20: Enzymes. Br J Pharmacol. 176 Issue S1: S297-S396.