ACP2

This gene encodes the beta subunit of lysosomal acid phosphatase (LAP). LAP is chemically and genetically distinct from red cell acid phosphatase. The encoded protein belongs to a family of distinct isoenzymes which hydrolyze orthophosphoric monoesters to alcohol and phosphate. Mutations in this gene or in the related alpha subunit gene cause acid phosphatase deficiency. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq]

Full Name

acid phosphatase 2, lysosomal

Biological Process

Dephosphorylation
Lysosome organization

Cellular Location

Lysosome membrane; Lysosome lumen. The soluble form arises by proteolytic processing of the membrane-bound form.

Topology

Lumenal: 31-380 aa
Helical: 381-401 aa
Cytoplasmic: 402-423 aa

PTM

The membrane-bound form is converted to the soluble form by sequential proteolytic processing. First, the C-terminal cytoplasmic tail is removed. Cleavage by a lysosomal protease releases the soluble form in the lysosome lumen.
N-glycosylated. The intermediates formed during enzymatic deglycosylation suggest that all eight predicted N-glycosylation sites are used.