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Iduronate 2-Sulfatase (F5F9E) Rabbit mAb

Iduronate 2-Sulfatase (F5F9E) Rabbit mAb #86051

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Supporting Data

REACTIVITY	H M R
SENSITIVITY	Endogenous
MW (kDa)	80
Source/Isotype	Rabbit IgG

Application Key:

WB-Western Blotting

Species Cross-Reactivity Key:

H-Human
M-Mouse
R-Rat

Product Information

Product Usage Information

Application	Dilution
Western Blotting	1:1000

Storage

Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

Protocol

Specificity / Sensitivity

Iduronate 2-Sulfatase (F5F9E) Rabbit mAb recognizes endogenous levels of total iduronate 2-sulfatase protein.

Species Reactivity:

Human, Mouse, Rat

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro160 of human iduronate 2-sulfatase protein.

Background

Iduronate 2-sulfatase (IDS) is a lysosomal enzyme expressed in pancreatic islets responsible for proteoglycan degradation, such as dermatan sulfate and heparan sulfate. It has a role in glucose-stimulated insulin secretion via a mechanism involving exocytosis activation through phosphorylation of protein kinase C alpha (PKCα) and its Myristoylated Alanine-Rich C-Kinase Substrate (MARCKS) (1). IDS was shown to be glycosylated in the endoplasmic reticulum (ER) and Golgi apparatus and proteolytically cleaved to generate the mature forms in the Golgi apparatus. Mature IDS is then translocated to the lysosome (2). Mutations in the IDS gene cause the progressive disease mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, an X-linked recessive lysosomal storage disorder (2-4). Inactivity, insufficiency, or absence of IDS leads to widespread accumulation of proteoglycans and subsequent lysosomal dysfunction in multiple organs and tissues, including the central nervous system (CNS) (4).

Database Links

UniProt ID: P22304

Entrez-Gene Id: 3423

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For Research Use Only. Not for Use in Diagnostic Procedures.

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