Thermo Fisher Scientific GNS Monoclonal Antibody (7D5H8H11)

Applications

Tested Dilution

Publications

ELISA (ELISA)

1:1,000-1:2,000

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG2b

Class

Monoclonal

Type

Antibody

Clone

7D5H8H11

Immunogen

Human cell-derived rhGNS

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Protein A

Storage buffer

PBS

Contains

no preservative

Storage conditions

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2786192

Product Specific Information

This product is preservative free. It is recommended to add sodium azide to avoid contamination (final concentration 0.05%-0.1%).

This antibody has specificity for Human GNS/G6S.

Target Information

GNS is a lysosomal enzyme found in all cells. It is involved in the catabolism of heparin, heparan sulphate, and keratan sulphate. Deficiency of this enzyme results in the accumulation of undegraded substrate and the lysosomal storage disorder mucopolysaccharidosis type IIID (Sanfilippo D syndrome). Mucopolysaccharidosis type IIID is the least common of the four subtypes of Sanfilippo syndrome.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.