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Overview
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Background
LONP2 (lon peptidase 2, peroxisomal), also known as LONP, LONPL or peroxisomal lon protease, is an 852 amino acid protein belonging to the peptidase S16 family. LONP2 assists in import of peroxisomal matrix proteins and is required for PTS2 (type 2 peroxisome targeting signal)-containing protein processing. As an ATP-dependent serine protease, LONP2 plays a role in degradation of polypeptides that are unassembled or incorrectly folded. The gene encoding LONP2 maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome. The GAN gene is located on chromosome 16 and, with mutation, may lead to giant axonal neuropathy, a nervous system disorder characterized by increasing malfunction with growth. The rare disorder Rubinstein-Taybi syndrome is also associated with chromosome 16, as is Crohn’s disease, which is a gastrointestinal inflammatory condition.
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