beta Mannosidase (beta-Manase), ELISA Kit

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

Store all reagents at 2-8 degree C.

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Small volumes of MANBA elisa kit 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.

MBS2608670 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the beta Mannosidase (beta-Manase) (MANBA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing MANBA. The ELISA analytical biochemical technique of the MBS2608670 kit is based on MANBA antibody-MANBA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect MANBA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, MANBA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

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MANBA_HUMAN

This gene encodes a member of the glycosyl hydrolase 2 family. The encoded protein localizes to the lysosome where it is the final exoglycosidase in the pathway for N-linked glycoprotein oligosaccharide catabolism. Mutations in this gene are associated with beta-mannosidosis, a lysosomal storage disease that has a wide spectrum of neurological involvement. [provided by RefSeq, Jul 2008]

MANBA: Exoglycosidase that cleaves the single beta-linked mannose residue from the non-reducing end of all N-linked glycoprotein oligosaccharides. Defects in MANBA are the cause of a mild disorder that affects peripheral and central nervous system myelin. Defects in MANBA are the cause of lysosomal beta- mannosidosis (LYSBMAN). Beta-mannosidosis is an autosomal recessive lysosomal storage disease of glycoprotein catabolism. Clinical features are heterogeneous with a wide range of symptoms and age of onset. The disease is associated with a range of neurological involvement, including various degrees of mental retardation in most of the cases, hearing loss and speech impairment, hypotonia, epilepsy and peripheral neuropathy. Affected individuals have a profound reduction in beta-mannosidase activity in plasma, fibroblasts and leukocytes. Belongs to the glycosyl hydrolase 2 family.

Protein type: EC 3.2.1.25; Glycan Metabolism - other glycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 4q24

Cellular Component: intracellular membrane-bound organelle; lysosomal lumen

Molecular Function: beta-mannosidase activity

Biological Process: carbohydrate metabolic process; oligosaccharide catabolic process

Disease: Mannosidosis, Beta A, Lysosomal

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