FLJ51211; WFRS; WFS; WOLFRAMIN

For Research Use Only. Not for use in diagnostic procedures.

IgG

Rabbit

Affinity purification

Store at-20 degree C or-80 degree C. Avoid freeze/thaw cycles.

Small volumes of anti-WFS1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Wolfram syndrome protein (WFS1) is an 890 amino acid protein that contains a cytoplasmic N-terminal domain, followed by nine-transmembrane domains and a luminal C-terminal domain. WFS1 is predominantly localized to the endoplasmic reticulum (ER) (1) and its expression is induced in response to ER stress, partially through transcriptional activation (2, 3). Research studies have shown that mutations in the WFS1 gene lead to Wolfram syndrome, an autosomal recessive neurodegenerative disorder defined by young-onset, non-immune, insulin-dependent diabetes mellitus and progressive optic atrophy (4).

Autophagy antibody; Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:500-1:2000
IHC: 1:50-1:200

Western blot analysis of extracts of mouse braincell lines, using WFS1 antibody.

100kDa

wolframin ER transmembrane glycoprotein

wolframin

Wolframin

This gene encodes a transmembrane protein, which is located primarily in the endoplasmic reticulum and ubiquitously expressed with highest levels in brain, pancreas, heart, and insulinoma beta-cell lines. Mutations in this gene are associated with Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness), an autosomal recessive disorder. The disease affects the brain and central nervous system. Mutations in this gene can also cause autosomal dominant deafness 6 (DFNA6), also known as DFNA14 or DFNA38. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2009]

Participates in the regulation of cellular Ca2+ homeostasis, at least partly, by modulating the filling state of the endoplasmic reticulum Ca2+ store.

1. Takeda, K. et al. (2001) Hum Mol Genet 10, 477-84. 2. Yamaguchi, S. et al. (2004) Biochem Biophys Res Commun 325, 250-6. 3. Ueda, K. et al. (2005) Eur J Endocrinol 153, 167-76. 4. Inoue, H. et al. (1998) Nat Genet 20, 143-8.

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