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Rabbit Anti-SGSH Recombinant Antibody (BA0166) (CBMAB-0826CQ)


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Summary

Host Animal
Rabbit
Specificity
Human
Clone
BA0166
Antibody Isotype
IgG
Application
IF, WB

Basic Information

Immunogen
Human SGSH aa 250 to the C-terminus
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
N-Sulfoglucosamine Sulfohydrolase
Introduction
This gene encodes the enzyme sulfamidase; one of several enzymes involved in the lysosomal degradation of heparan sulfate. Catalyzes a step in lysosomal heparan sulfate degradation.
Entrez Gene ID
6448
UniProt ID
P51688
Alternative Names
HSS; SFMD; MPS3A
Function
Catalyzes a step in lysosomal heparan sulfate degradation.
Biological Process
Biological Process glycosaminoglycan catabolic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process heparan sulfate proteoglycan catabolic processManual Assertion Based On ExperimentIMP:UniProtKB
Cellular Location
Lysosome
Involvement in disease
Mucopolysaccharidosis 3A (MPS3A):
A severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.
PTM
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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