Cell Biology Antibodies 12
Anti-MAN2B1 Antibody (CAB9937)
- SKU:
- CAB9937
- Product Type:
- Antibody
- Applications:
- WB
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Description
Antibody Name: | Anti-MAN2B1 Antibody |
Antibody SKU: | CAB9937 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB |
Reactivity: | Mouse |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 640-850 of human MAN2B1 (NP_000519.2). |
Application: | WB |
Recommended Dilution: | WB 1:500 - 1:2000 |
Reactivity: | Mouse |
Positive Samples: | Mouse liver |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 640-850 of human MAN2B1 (NP_000519.2). |
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: | TFFW YNAS IGDN ESDQ ASGA YIFR PNQQ KPLP VSRW AQIH LVKT PLVQ EVHQ NFSA WCSQ VVRL YPGQ RHLE LEWS VGPI PVGD TWGK EVIS RFDT PLET KGRF YTDS NGRE ILER RRDY RPTW KLNQ TEPV AGNY YPVN TRIY ITDG NMQL TVLT DRSQ GGSS LRDG SLEL MVHR RLLK DDGR GVSE PLME NGSG AWVR GRHL VLLD TAQ |
Gene ID: | 4125 |
Uniprot: | O00754 |
Cellular Location: | Lysosome |
Calculated MW: | 113kDa |
Observed MW: | 170kDa |
Synonyms: | MAN2B1, LAMAN, MANB |
Background: | 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. |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Glycan Metabolism - other glycan degradation; Hydrolase; EC 3.2.1.24 Chromosomal Location of Human Ortholog: 19p13.2 Cellular Component: lysosome Molecular Function:zinc ion binding; carbohydrate binding; alpha-mannosidase activity Biological Process: mannose metabolic process; protein deglycosylation; protein modification process Disease: Mannosidosis, Alpha B, Lysosomal |
NCBI Summary: | 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] |
UniProt Code: | O00754 |
NCBI GenInfo Identifier: | 51873064 |
NCBI Gene ID: | 4125 |
NCBI Accession: | NP_000519.2 |
UniProt Secondary Accession: | O00754,O15330, Q16680, Q93094, Q9BW13, G5E928, |
UniProt Related Accession: | O00754 |
Molecular Weight: | 113,744 Da |
NCBI Full Name: | lysosomal alpha-mannosidase isoform 1 |
NCBI Synonym Full Names: | mannosidase, alpha, class 2B, member 1 |
NCBI Official Symbol: | MAN2B1 |
NCBI Official Synonym Symbols: | MANB; LAMAN |
NCBI Protein Information: | lysosomal alpha-mannosidase; mannosidase alpha-B; mannosidase, alpha B, lysosomal; lysosomal acid alpha-mannosidase |
UniProt Protein Name: | Lysosomal alpha-mannosidase |
UniProt Synonym Protein Names: | Lysosomal acid alpha-mannosidase; Mannosidase alpha class 2B member 1; Mannosidase alpha-BCleaved into the following 5 chains: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 |
UniProt Gene Name: | MAN2B1 |
UniProt Entry Name: | MA2B1_HUMAN |