Guinea Pig beta Mannosidase (beta-Manase) ELISA Kit | MANBA elisa kit
Guinea pig beta Mannosidase (beta-Manase) ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
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: Hydrolase; Glycan Metabolism - other glycan degradation; EC 3.2.1.25
Chromosomal Location of Human Ortholog: 4q24
Cellular Component: intracellular membrane-bound organelle; lysosome
Molecular Function: beta-mannosidase activity
Biological Process: mannan catabolic process; carbohydrate metabolic process; protein modification process; glycoprotein catabolic process
Disease: Mannosidosis, Beta A, Lysosomal
Research Articles on MANBA
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Product Notes
The Guinea Pig MANBA manba (Catalog #AAA260697) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA260697 ELISA Kit recognizes Guinea Pig MANBA. It is sometimes possible for the material contained within the vial of "beta Mannosidase (beta-Manase), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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