Rabbit Anti-SGSH Recombinant Antibody (CBXS-1436) (CBMAB-S4294-CQ)

Name: Rabbit Anti-SGSH Recombinant Antibody (CBXS-1436)
SKU: CBMAB-S4294-CQ
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Rabbit

Clone

CBXS-1436

Application

WB, IF

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!]

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

More Infomation

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. Mutations in this gene are associated with the lysosomal storage disease mucopolysaccaridosis IIIA, also known as Sanfilippo syndrome A, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.

Entrez Gene ID

6448

UniProt ID

P51688

Alternative Names

N-Sulfoglucosamine Sulfohydrolase; Mucopolysaccharidosis Type IIIA; Sulfoglucosamine Sulfamidase; Sulphamidase; HSS; N-Sulphoglucosamine Sulphohydrolase;

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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Isotype control

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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