ACP2 Antibody (PACO18512)
- SKU:
- PACO18512
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | ACP2 Antibody (PACO18512) |
Antibody SKU: | PACO18512 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB |
Recommended Dilutions: | ELISA:1:1000-1:2000, WB:1:200-1:1000 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | Synthetic peptide of human ACP2 |
Form: | Liquid |
Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Purification Method: | Antigen affinity purification |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-2: 293T cells, hela cells, Primary antibody: PACO18512(ACP2 Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 3 minutes. |
Background: | This gene encodes the β 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 α subunit gene cause acid, phosphatase deficiency. Multiple alternatively spliced transcript variants encoding different isoforms have been identified for this gene. |
Synonyms: | acid, phosphatase 2, lysosomal |
UniProt Protein Function: | 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] |
UniProt Code: | P11117 |
NCBI GenInfo Identifier: | 197100230 |
NCBI Gene ID: | 53 |
NCBI Accession: | NP_001124536.1 |
UniProt Secondary Accession: | P11117,Q561W5, Q9BTU7, E9PCI1 |
UniProt Related Accession: | P11117 |
Molecular Weight: | 18,417 Da |
NCBI Full Name: | |
NCBI Synonym Full Names: | acid phosphatase 2, lysosomal |
NCBI Official Symbol: | ACP2 |
NCBI Official Synonym Symbols: | LAP |
NCBI Protein Information: | lysosomal acid phosphatase |
UniProt Protein Name: | Lysosomal acid phosphatase |
UniProt Synonym Protein Names: | |
Protein Family: | Leucine aminopeptidase |
UniProt Gene Name: | ACP2 |
UniProt Entry Name: | PPAL_HUMAN |
Antibodies |
ACP2 Antibody (PACO07566) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
Recommended Products |
Anti-FITC Goat Anti-Rabbit IgG (H+L) Antibody (CABS011) |
Anti-HRP-conjugated Beta Actin Antibody (CABC028) |