ACP2
Involvement in disease
Lysosomal acid phosphatase has been shown to be deficient in cultured fibroblasts from patients manifesting intermittent vomiting, hypotonia, lethargy, opisthotonos, terminal bleeding and death in early infancy.
Post-translational modifications
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.
Sequence Similarities
Belongs to the histidine acid phosphatase family.
Cellular localization
- Lysosome membrane
- Single-pass membrane protein
- Lumenal side
- Lysosome lumen
- The soluble form arises by proteolytic processing of the membrane-bound form.
Alternative names
Lysosomal acid phosphatase, LAP, ACP2