Reactome | LONP1 degrades mitochondrial inner membrane proteins

LONP1 degrades mitochondrial inner membrane proteins

Stable Identifier

R-HSA-9838004

Type

Reaction [uncertain]

Species

Homo sapiens

Compartment

mitochondrial inner membrane

ReviewStatus

5/5

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LONP1 degrades mitochondrial inner membrane proteins

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After binding a substrate protein, LONP1 degrades the substrate protein using an ATP-dependent mechanism in which ATP hydrolysis causes translocation of the substrate protein to the protease site (Ambro et al. 2014, Mohammed et al. 2022). LONP1 predominantly cleaves peptide bonds adjacent to hydrophobic amino acid residues (He et al. 2018). LONP1 binds and degrades several mitochondrial inner membrane proteins (Ondrovicova et al. 2005, Lee et al. 2021), including the mitochondrial processing peptidase subunit PMPCA (Ondrovicova et al. 2005).

Literature References

PubMed ID	Title	Journal	Year
24520911	Mutations to a glycine loop in the catalytic site of human Lon changes its protease, peptidase and ATPase activities Ambro, Ľ, Pevala, V, Ondrovičová, G, Bellová, J, Kunová, N, Kutejova, E, Bauer, J	FEBS J	2014
30120231	Multiple domains of bacterial and human Lon proteases define substrate selectivity He, L, Luo, D, Yang, F, Li, C, Zhang, X, Deng, H, Zhang, JR	Emerg Microbes Infect	2018
33637676	LONP1 and ClpP cooperatively regulate mitochondrial proteostasis for cancer cell survival Lee, YG, Kim, HW, Nam, Y, Shin, KJ, Lee, YJ, Park, DH, Rhee, HW, Seo, JK, Chae, YC	Oncogenesis	2021
15870080	Cleavage site selection within a folded substrate by the ATP-dependent lon protease Ondrovicová, G, Liu, T, Singh, K, Tian, B, Li, H, Gakh, O, Perecko, D, Janata, J, Granot, Z, Orly, J, Kutejova, E, Suzuki, CK	J Biol Chem	2005
35870450	Catalytic cycling of human mitochondrial Lon protease Mohammed, I, Schmitz, KA, Schenck, N, Balasopoulos, D, Topitsch, A, Maier, T, Abrahams, JP	Structure	2022