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MAN2B1 Rabbit pAb

MAN2B1 Rabbit pAb

     
  • 14 - MAN2B1 Rabbit pAb AP57198
    Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (MAN2B1) Polyclonal Antibody, Unconjugated (AP57198) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
IHC-P, IHC-F, IF
Primary Accession O00754
Reactivity Mouse
Predicted Rat, Pig, Dog, Human, Rabbit
Host Rabbit
Clonality Polyclonal
Calculated MW 113744 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human MAN2B1
Epitope Specificity 151-250/1011
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Lysosome.
DISEASE Mannosidosis, alpha B, lysosomal (MANSA) [MIM:248500]: A lysosomal storage disease characterized by accumulation of unbranched oligosaccharide chains. This accumulation is expressed histologically as cytoplasmic vacuolation predominantly in the CNS and parenchymatous organs. Depending on the clinical findings at the age of onset, a severe infantile (type I) and a mild juvenile (type II) form of alpha-mannosidosis are recognized. There is considerable variation in the clinical expression with mental retardation, recurrent infections, impaired hearing and Hurler-like skeletal changes being the most consistent abnormalities. Note: The disease is caused by mutations affecting the gene represented in this entry.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This gene encodes an enzyme that hydrolyzes terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. Its activity is necessary for the catabolism of N-linked carbohydrates released during glycoprotein turnover and it is member of family 38 of glycosyl hydrolases. The full length protein is processed in two steps. First, a 49 aa leader sequence is cleaved off and the remainder of the protein is processed into 3 peptides of 70 kDa, 42 kDa (D) and 13/15 kDa (E). Next, the 70 kDa peptide is further processed into three peptides (A, B and C). The A, B and C peptides are disulfide-linked. Defects in this gene have been associated with lysosomal alpha-mannosidosis. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2010]
Additional Information
Gene ID 4125
Other Names Lysosomal alpha-mannosidase, Laman, 3.2.1.24, Lysosomal acid alpha-mannosidase, Mannosidase alpha class 2B member 1, Mannosidase alpha-B, Lysosomal alpha-mannosidase A peptide, Lysosomal alpha-mannosidase B peptide, Lysosomal alpha-mannosidase C peptide, Lysosomal alpha-mannosidase D peptide, Lysosomal alpha-mannosidase E peptide, MAN2B1 (HGNC:6826), LAMAN, MANB
Dilution IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name MAN2B1 (HGNC:6826)
Synonyms LAMAN, MANB
Function Can hydrolyze a variety of glycan substrates containing terminal alpha-mannosidic linkages. Cleaves alpha 1,2-, alpha 1,3-, and alpha 1,6-linked mannose residues on oligosaccharides generated by N- glycoprotein degradation pathways.
Cellular Location Lysosome lumen. Secreted. Note=The protein may be secreted in an unprocessed but enzymatically active form (PubMed:11350179, PubMed:21505070). Secreted enzyme may enter cells via endocytosis (PubMed:11350179).
Research Areas

BACKGROUND

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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