Metabolism Antibodies 2

Anti-LIPA Antibody (CAB6385)

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SKU:
CAB6385
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-LIPA Antibody
Antibody SKU:CAB6385
Antibody Size:20uL, 50uL, 100uL
Application:WB IHC
Reactivity:Human, Rat
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 110-399 of human LIPA (NP_001121077.1).
Application:WB IHC
Recommended Dilution:WB 1:500 - 1:2000 IHC 1:50 - 1:200
Reactivity:Human, Rat
Positive Samples:MCF7, SW620, 293T, Rat liver
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 110-399 of human LIPA (NP_001121077.1).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:DAGF DVWM GNSR GNTW SRKH KTLS VSQD EFWA FSYD EMAK YDLP ASIN FILN KTGQ EQVY YVGH SQGT TIGF IAFS QIPE LAKR IKMF FALG PVAS VAFC TSPM AKLG RLPD HLIK DLFG DKEF LPQS AFLK WLGT HVCT HVIL KELC GNLC FLLC GFNE RNLN MSRV DVYT THSP AGTS VQNM LHWS QAVK FQKF QAFD WGSS AKNY FHYN QSYP PTYN VKDM LVPT AVWS GGHD WLAD VYDV NILL TQIT NLVF HESI PEWE HLDF IWGL DAPW RLYN KIIN LMRK YQ
Gene ID:3988
Uniprot:P38571
Cellular Location:Lysosome
Calculated MW:39kDa/45kDa
Observed MW:40-45kDa
Synonyms:LIPA, CESD, LAL, lipase A
Background:This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced transcript variants have been found for this gene.
UniProt Protein Function:LIPA: Crucial for the intracellular hydrolysis of cholesteryl esters and triglycerides that have been internalized via receptor- mediated endocytosis of lipoprotein particles. Important in mediating the effect of LDL (low density lipoprotein) uptake on suppression of hydroxymethylglutaryl-CoA reductase and activation of endogenous cellular cholesteryl ester formation. Defects in LIPA are the cause of Wolman disease (WOD). WOD is a severe manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. WOD occurs in infancy and is nearly always fatal before the age of 1 year. Defects in LIPA are the cause of cholesteryl ester storage disease (CESD). CESD is a mild manifestation of LIPA deficiency, leading to the accumulation of cholesteryl esters and triglycerides in most tissues of the body. It is characterized by late-onset. Belongs to the AB hydrolase superfamily. Lipase family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Lipid Metabolism - steroid biosynthesis; Hydrolase; EC 3.1.1.13

Chromosomal Location of Human Ortholog: 10q23.2-q23.3

Cellular Component: lysosome

Molecular Function:sterol esterase activity; lipase activity

Biological Process: cell proliferation; homeostasis of number of cells within a tissue; tissue remodeling; cell morphogenesis; cytokine production; inflammatory response; lipid catabolic process; lung development

Disease: Lysosomal Acid Lipase Deficiency

NCBI Summary:This gene encodes lipase A, the lysosomal acid lipase (also known as cholesterol ester hydrolase). This enzyme functions in the lysosome to catalyze the hydrolysis of cholesteryl esters and triglycerides. Mutations in this gene can result in Wolman disease and cholesteryl ester storage disease. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jan 2014]
UniProt Code:P38571
NCBI GenInfo Identifier:68067636
NCBI Gene ID:3988
NCBI Accession:P38571.2
UniProt Related Accession:P38571
Molecular Weight:
NCBI Full Name:Lysosomal acid lipase/cholesteryl ester hydrolase
NCBI Synonym Full Names:lipase A, lysosomal acid type
NCBI Official Symbol:LIPA  
NCBI Official Synonym Symbols:LAL; CESD  
NCBI Protein Information:lysosomal acid lipase/cholesteryl ester hydrolase
UniProt Protein Name:Lysosomal acid lipase/cholesteryl ester hydrolase
UniProt Synonym Protein Names:Cholesteryl esterase; Lipase A; Sterol esterase
UniProt Gene Name:LIPA  
UniProt Entry Name:LICH_HUMAN
Anti-LIPA Antibody (CAB6385)Western blot analysis of extracts of various cell lines, using LIPA antibody (CAB6385) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 90s.
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