DFNA14; DFNA38; DFNA6; DIDMOAD; WFRS; WFS; Wolframin

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

This assay has high sensitivity and excellent specificity for detection of Wolfram Syndrome Protein 1 (WFS1).
No significant cross-reactivity or interference between Wolfram Syndrome Protein 1 (WFS1) and analogues was observed.

The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition.
To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of WFS1 clia kit 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.

The microplate provided in this kit has been pre-coated with an antibody specific to Wolfram Syndrome Protein 1 (WFS1). Standards or samples are then added to the appropriate microplate wells with a biotin-conjugated antibody specific to Wolfram Syndrome Protein 1 (WFS1). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. Then the mixture of substrate A and B is added to generate glow light emission kinetics. Upon plate development, the intensity of the emitted light is proportional to the Wolfram Syndrome Protein 1 (WFS1) level in the sample or standard.;

Neuroscience

100,292 Da

Wolfram syndrome 1 (wolframin)

wolframin

Wolframin

WFS1_HUMAN

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]

WFS1: Participates in the regulation of cellular Ca(2+) homeostasis, at least partly, by modulating the filling state of the endoplasmic reticulum Ca(2+) store. Highly expressed in heart followed by brain, placenta, lung and pancreas. Weakly expressed in liver, kidney and skeletal muscle. Also expressed in islet and beta-cell insulinoma cell line.

Protein type: Endoplasmic reticulum; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 4p16.1

Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; dendrite; integral to endoplasmic reticulum membrane

Molecular Function: calmodulin binding; transcription activator binding; transporter activity; ubiquitin protein ligase binding; calcium-dependent protein binding; ATPase binding

Biological Process: ER-associated protein catabolic process; protein maturation via protein folding; protein stabilization; olfactory behavior; positive regulation of proteolysis; unfolded protein response; negative regulation of transcription factor activity; glucose homeostasis; positive regulation of protein metabolic process; neurological system process; calcium ion homeostasis; endoplasmic reticulum calcium ion homeostasis; unfolded protein response, activation of signaling protein activity; sensory perception of sound; cellular protein metabolic process; visual perception; positive regulation of protein ubiquitination; negative regulation of programmed cell death; renal water homeostasis; negative regulation of neuron apoptosis; kidney development; positive regulation of calcium ion transport; positive regulation of growth; ER overload response

Disease: Deafness, Autosomal Dominant 6; Wolfram Syndrome 1; Wolfram-like Syndrome, Autosomal Dominant; Cataract 41; Diabetes Mellitus, Noninsulin-dependent

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