Human ACP2 Recombinant Protein (RPPB5777)

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SKU:
RPPB5777
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
ACP2
Synonyms:
Acid Phosphatase 2
Synonyms:
Lysosoma
Synonyms:
EC 3132 LAP
Synonyms:
Lysosomal Acid Phosphatase
Source:
Escherichia Coli
Uniprot:
P11117
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Description

Product Name:Human ACP2 Recombinant Protein
Product Code:RPPB5777
Size:20µg
Species:Human
Target:ACP2
Synonyms:Acid Phosphatase 2, Lysosoma, EC 3.1.3.2 LAP, Lysosomal Acid Phosphatase, ACP2.
Source:Escherichia Coli
Physical Appearance:Sterile filtered colorless solution.
Formulation:ACP2 protein solution (1mg/ml) contains 20mM Tris-HCl (pH8.0) and 10% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 85.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSRSLRFVT LLYRHGDRSP VKTYPKDPYQ EEEWPQGFGQ LTKEGMLQHW ELGQALRQRY HGFLNTSYHR QEVYVRSTDF DRTLMSAEAN LAGLFPPNGM QRFNPNISWQ PIPVHTVPIT EDRLLKFPLG PCPRYEQLQN ETRQTPEYQN ESSRNAQFLD MVANETGLTD LTLETVWNVY DTLFCEQTHG LRLPPWASPQ TMQRLSRLKD FSFRFLFGIY QQAEKARLQG GVLLAQIRKN LTLMATTSQL PKLLVYSAHD TTLVALQMAL DVYNGEQAPY ASCHIFELYQ EDSGNFSVEM YFRNESDKAP WPLSLPGCPH RCPLQDFLRL TEPVVPKDWQ QECQLASGPA DTE

Acid Phosphatase-2, alsoknown as ACP2 is composed of two subunits, Alpha & beta, and is chemically as well as genetically distinct fromred cell acid phosphatase. ACP2 belongs to a family of distinct isoenzymeswhich hydrolyze orthophosphoric monoesters to alcohol and phosphate. Inaddition, Acid phosphatase deficiency is caused by mutations in the ACP2-beta subunit as well asACP3-alpha subunit genes.

ACP2 Human Recombinant produced in E.Coli is a single, non-glycosylatedpolypeptide chain containing 373 amino acids (31-380 a.a.) and having amolecular mass of 42.9kDa. ACP2 is fused to a 23 amino acid His-Tag at N-Terminusand purified by conventional chromatography techniques.

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
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