For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against MAN2B1. It has beenselected for its ability to recognize MAN2B1 in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-MAN2B1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.2-2ug/mL;1:250-2500
Immunohistochemistry: 5-20ug/mL;1:25-100
Immunocytochemistry: 5-20ug/mL;1:25-100
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant protein.

DABstainingonIHC-P.Samples:HumanTissue)

113,616 Da

mannosidase alpha class 2B member 1

lysosomal alpha-mannosidase

Lysosomal alpha-mannosidase

LAMAN; MANB; Laman??[Similar Products]

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]

MAN2B1: Necessary for the catabolism of N-linked carbohydrates released during glycoprotein turnover. Cleaves all known types of alpha-mannosidic linkages. Defects in MAN2B1 are the cause of lysosomal alpha- mannosidosis (AM). AM is 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. Belongs to the glycosyl hydrolase 38 family.

Protein type: EC 3.2.1.24; Glycan Metabolism - other glycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 19p13.13

Cellular Component: extracellular region; extracellular space; lysosomal lumen

Molecular Function: alpha-mannosidase activity; carbohydrate binding; metal ion binding; protein binding

Biological Process: cellular protein modification process; mannose metabolic process; neutrophil degranulation; oligosaccharide catabolic process; protein deglycosylation

Disease: Mannosidosis, Alpha B, Lysosomal

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