SGSH Antibody

Product Specifications

Background

SGSH is one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.

NCBI Gene ID

6448

Swiss Prot

P51688

Accession Number

P51688

Host

Rabbit

Reactivity

Human

Immunogen

This SGSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 420-449 amino acids from the C-terminal region of human SGSH.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Neuroscience, Signal Transduction

Purification

This antibody is purified through a protein A column, followed by peptide affinity purification.

Concentration

Batch dependent

Buffer

Supplied in PBS with 0.09% (W/V) sodium azide.

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Calculated Molecular Weight

57 kDa

Fragment

Rabbit Ig

Applications Notes

For WB starting dilution is: 1:4000

Symbol

SGSH

NCBI Official Name

N-sulphoglucosamine sulphohydrolase

NCBI Organism

Homo sapiens

Background Reference 01

Sleat, D.E., et al. Mol. Cell Proteomics 5 (4) :686-701 (2006)

Background Reference 02

Bekri, S., et al. J. Inherit. Metab. Dis. 28 (4) :601-602 (2005)

Background Reference 03

Muschol, N., et al. Hum. Mutat. 23 (6) :559-566 (2004)

Background Reference 04

Zhang, H., et al. Nat. Biotechnol. 21 (6) :660-666 (2003)

Other Product Names

N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH, HSS

Tested Applications

Physical Properties

Liquid

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