The ACP2 Polyclonal Antibody (PACO7566) is a valuable tool for researchers studying the ACP2 protein, an enzyme involved in lysosomal degradation processes. This antibody, produced in rabbits, exhibits high reactivity with human samples and has been validated for use in Western blot applications. By specifically binding to the ACP2 protein, this antibody enables precise detection and analysis in a variety of cell types, making it an essential reagent for studies in cell biology and lysosomal function.ACP2, also known as acid phosphatase 2, plays a crucial role in maintaining cellular homeostasis by breaking down various molecules within lysosomes. Dysregulation of ACP2 activity has been linked to lysosomal storage disorders and other diseases, highlighting its importance in cellular function.
Research into ACP2 function is essential for understanding lysosomal biology and exploring potential therapeutic strategies for related disorders.Overall, the ACP2 Polyclonal Antibody is a reliable tool for scientists investigating lysosomal degradation pathways and seeking a deeper understanding of ACP2's role in cellular physiology. Its high specificity and reactivity make it an excellent choice for experiments aimed at elucidating the function and regulation of the ACP2 enzyme in health and disease.
ACP2: Defects in ACP2 are a cause of acid phosphatase deficiency (ACPHD). The clinical features are intermittent vomiting, hypotonia, lethargy, opisthotonos, terminal bleeding, and death in early infancy. Lysosomal acid phosphatase is deficient in cultured fibroblasts and multiple tissues. Belongs to the histidine acid phosphatase family.Protein type: Membrane protein, integral; Phosphatase (non-protein); Cofactor and Vitamin Metabolism - riboflavin; EC 3.1.3.2; Motility/polarity/chemotaxisChromosomal Location of Human Ortholog: 11p11.2|11p12-p11Cellular Component: lysosomal lumen; membrane; lysosome; lysosomal membrane; integral to membraneMolecular Function: acid phosphatase activityBiological Process: dephosphorylation; lysosome organization and biogenesis; skeletal developmentDisease: Acid Phosphatase Deficiency
UniProt Protein Details:
NCBI Summary:
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. LAP-deficiencies in mice cause multiple defects including bone structure alterations, lysosomal storage defects in the kidneys and central nervous system, and an increased tendency towards seizures. An enzymatically-inactive allele of LAP in mice exhibited a more severe phenotype than the null allele, and defects included cerebellum abnormalities, growth retardation, hair-follicle abnormalities, and an ataxia-like phenotype. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Oct 2014]
