AUNB1; DFNB6; DFNB9; FER1L2; NSRD9; OTTHUMP00000122455

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

This assay has high sensitivity and excellent specificity for detection of Rat OTOF. No significant cross-reactivity or interference between Rat OTOF and analogues was observed.

Store at 2-8 degree C.

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Small volumes of OTOF elisa 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.

MBS285349 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Otoferlin, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing OTOF. The ELISA analytical biochemical technique of the MBS285349 kit is based on OTOF antibody-OTOF antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect OTOF antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, OTOF. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Principle of the Assay: This assay employs a two-site sandwich ELISA to quantitate OTOF in samples. An antibody specific for OTOF has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyOTOF present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for OTOF is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of OTOF bound in the initial step. The color development is stopped and the intensity of the color is measured.

226,339 Da

otoferlin

otoferlin

Otoferlin

Fer1l2??[Similar Products]

human homolog is associated with an autosomal recessive form of prelingual sensorineural deafness, DFNB9 [RGD, Feb 2006]

OTOF: Key calcium ion sensor involved in the Ca(2+)-triggered synaptic vesicle-plasma membrane fusion and in the control of neurotransmitter release at these output synapses. Interacts in a calcium-dependent manner to the presynaptic SNARE proteins at ribbon synapses of cochlear inner hair cells (IHCs) to trigger exocytosis of neurotransmitter. Also essential to synaptic exocytosis in immature outer hair cells (OHCs). May also play a role within the recycling of endosomes. Defects in OTOF are the cause of deafness autosomal recessive type 9 (DFNB9). DFNB9 is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. Defects in OTOF are a cause of auditory neuropathy, autosomal recessive, type 1 (AUNB1). A form of sensorineural hearing loss with absent or severely abnormal auditory brainstem response, in the presence of normal cochlear outer hair cell function and normal otoacoustic emissions. Auditory neuropathies result from a lesion in the area including the inner hair cells, connections between the inner hair cells and the cochlear branch of the auditory nerve, the auditory nerve itself and auditory pathways of the brainstem. In some cases AUNB1 phenotype can be temperature sensitive. Belongs to the ferlin family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Vesicle

Chromosomal Location of Human Ortholog: 6q14

Cellular Component: apical part of cell; basolateral plasma membrane; cell junction; cytoplasm; endoplasmic reticulum; endoplasmic reticulum membrane; integral component of membrane; plasma membrane; presynaptic active zone membrane; synaptic vesicle membrane

Molecular Function: calcium ion binding; protein binding; protein complex binding

Biological Process: sensory perception of sound; synaptic vesicle exocytosis; synaptic vesicle priming

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