catalog number :
MBS926128
products type :
ELISA Kit
products full name :
Human Lysosomal alpha-mannosidase, MAN2B1 ELISA Kit
products short name :
mannosidase, alpha, class 2B, member 1
products name syn :
Human Lysosomal alpha-mannosidase (MAN2B1) ELISA kit; LAMAN; MANB; lysosomal acid alpha-mannosidase; mannosidase; alpha B; lysosomal; mannosidase; alpha; class 2B; member 1
other names :
lysosomal alpha-mannosidase isoform 1; Lysosomal alpha-mannosidase; lysosomal alpha-mannosidase; mannosidase alpha-B; mannosidase, alpha B, lysosomal; lysosomal acid alpha-mannosidase; mannosidase, alpha, class 2B, member 1; 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
products gene name :
MAN2B1
other gene names :
MAN2B1; MAN2B1; MANB; LAMAN; LAMAN; MANB; Laman
uniprot entry name :
MA2B1_HUMAN
specificity :
This assay has high sensitivity and excellent specificity for detection of human MAN2B1. No significant cross-reactivity or interference between human MAN2B1 and analogues was observed.
storage stability :
Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.
other info1 :
Samples: Serum, plasma, tissue homogenates, Cell lysates. Assay Type: Sandwich. Detection Range: 15.6 pg/ml -1000 pg/ml. Sensitivity: The minimum detectable dose of human MAN2B1 is typically less than 3.9 pg/ml. The sensitivity of this assay, or Lower Limit of Detection (LLD) was defined as the lowest protein concentration that could be differentiated from zero. It was determined the mean O.D value of 20 replicates of the zero standard added by their three standard deviations.
other info2 :
Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.
products description :
Priciple of the assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for MAN2B1 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any MAN2B1 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for MAN2B1 is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of MAN2B1 bound in the initial step. The color development is stopped and the intensity of the color is measured.
ncbi acc num :
NP_000519.2
ncbi gb acc num :
NM_000528.3
ncbi mol weight :
113,744 Da
ncbi pathways :
Lysosome Pathway (99052); Lysosome Pathway (96865); Other Glycan Degradation Pathway (82976); Other Glycan Degradation Pathway (346)
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 summary :
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: 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
size4 :
10x96-Strip-Wells