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Otoferlin antibody - 178 003 K.O.

Otoferlin is a potential Ca2+ sensor of transmitter release in hair cells of the inner ear
Rabbit polyclonal purified antibody
Cat. No.: 178 003
Amount: 50 µg
Price: $375.00
Cat. No. 178 003 50 µg specific antibody, lyophilized. Affinity purified with the immunogen. Albumin and azide were added for stabilization. For reconstitution add 50 µl H2O to get a 1mg/ml solution in PBS. Then aliquot and store at -20°C to -80°C until use.
Antibodies should be stored at +4°C when still lyophilized. Do not freeze!
Applications
 
WB: not tested yet
IP: not tested yet
ICC: not tested yet
IHC: 1 : 5000 gallery  
IHC-P: not tested yet
iDISCO: yes

Western blot (WB); separation of proteins by PAGE and subsequent transfer to a membrane. Detection of target molecules is carried out with antibodies. Some antibodies require special sample preparation steps. For details, please refer to the “Remarks” section.

Immunoprecipitation (IP); Immunoisolation or pulldown of a target molecule using an antibody. For details and product specific hints, please refer to the ”Remarks” section.

Immunocytochemistry (ICC) on 4% PFA fixed cells. Immunoreactivity is usually revealed by fluorescence. Some antibodies require special fixation methods. For details, please refer to the “Remarks” section.

Immunohistochemistry (IHC) on 4% PFA perfusion fixed tissue with 24h PFA post fixation. Immunoreactivity is usually revealed by fluorescence or a chromogenic substrate. Some antibodies require special fixation methods or antigen retrieval steps. For details, please refer to the ”Remarks” section.

Immunohistochemistry (IHC-P) of formalin fixed, paraffin embedded (FFPE) tissue (some antibodies require special antigen retrieval steps, please refer to the ”Remarks” section). Immunoreactivity is usually revealed by fluorescence or a chromogenic substrate.

iDISCO is a fast and cost-effective sample preparation method for clearing, immunolabeling and volume imaging of large biological samples. The clearing is carried out by benzyl-ether treatment. For additional experimental details, please refer to the „Remarks“ section or visit https://idisco.info.

Immunogen Synthetic peptide corresponding to AA 181 to 196 from mouse Otoferlin (UniProt Id: Q9ESF1-1)
Reactivity Reacts with: mouse (Q9ESF1).
Other species not tested yet.

Antigen identical in rat.
Specificity K.O. validated PubMed: 37248244
Data sheet 178_003.pdf

References for Otoferlin - 178 003

Cochlear transcript diversity and its role in auditory functions implied by an otoferlin short isoform.
Liu H, Liu H, Wang L, Song L, Jiang G, Lu Q, Yang T, Peng H, Cai R, Zhao X, Zhao T, et al.
Nature communications (2023) 141: 3085. 178 003 IHC; KO verified; tested species: mouse
Proteomic analysis reveals the composition of glutamatergic organelles of auditory inner hair cell.
Cepeda AP, Ninov M, Neef J, Parfentev I, Kusch K, Reisinger E, Jahn R, Moser T, Urlaub H
Molecular & cellular proteomics : MCP (2023) : 100704. 178 003 IHC; tested species: mouse
Overloaded Adeno-Associated Virus as a Novel Gene Therapeutic Tool for Otoferlin-Related Deafness.
Rankovic V, Vogl C, Dörje NM, Bahader I, Duque-Afonso CJ, Thirumalai A, Weber T, Kusch K, Strenzke N, Moser T
Frontiers in molecular neuroscience (2020) 13: 600051. 178 003 IHC, iDISCO; tested species: mouse
A new probe for super-resolution imaging of membranes elucidates trafficking pathways.
Revelo NH, Kamin D, Truckenbrodt S, Wong AB, Reuter-Jessen K, Reisinger E, Moser T, Rizzoli SO
The Journal of cell biology (2014) 2054: 591-606. 178 003 IHC; tested species: mouse
Overloaded Adeno-Associated Virus as a Novel Gene Therapeutic Tool for Otoferlin-Related Deafness.
Rankovic V, Vogl C, Dörje NM, Bahader I, Duque-Afonso CJ, Thirumalai A, Weber T, Kusch K, Strenzke N, Moser T
Frontiers in molecular neuroscience (2020) 13: 600051. 178 003 IHC, iDISCO; tested species: mouse
Cat. No.: 178 003
Amount: 50 µg
Price: $375.00
Cochlear transcript diversity and its role in auditory functions implied by an otoferlin short isoform.
Liu H, Liu H, Wang L, Song L, Jiang G, Lu Q, Yang T, Peng H, Cai R, Zhao X, Zhao T, et al.
Nature communications (2023) 141: 3085. 178 003 IHC; KO verified; tested species: mouse
Proteomic analysis reveals the composition of glutamatergic organelles of auditory inner hair cell.
Cepeda AP, Ninov M, Neef J, Parfentev I, Kusch K, Reisinger E, Jahn R, Moser T, Urlaub H
Molecular & cellular proteomics : MCP (2023) : 100704. 178 003 IHC; tested species: mouse
Overloaded Adeno-Associated Virus as a Novel Gene Therapeutic Tool for Otoferlin-Related Deafness.
Rankovic V, Vogl C, Dörje NM, Bahader I, Duque-Afonso CJ, Thirumalai A, Weber T, Kusch K, Strenzke N, Moser T
Frontiers in molecular neuroscience (2020) 13: 600051. 178 003 IHC, iDISCO; tested species: mouse
A new probe for super-resolution imaging of membranes elucidates trafficking pathways.
Revelo NH, Kamin D, Truckenbrodt S, Wong AB, Reuter-Jessen K, Reisinger E, Moser T, Rizzoli SO
The Journal of cell biology (2014) 2054: 591-606. 178 003 IHC; tested species: mouse
Overloaded Adeno-Associated Virus as a Novel Gene Therapeutic Tool for Otoferlin-Related Deafness.
Rankovic V, Vogl C, Dörje NM, Bahader I, Duque-Afonso CJ, Thirumalai A, Weber T, Kusch K, Strenzke N, Moser T
Frontiers in molecular neuroscience (2020) 13: 600051. 178 003 IHC, iDISCO; tested species: mouse
Background

Otoferlin is a transmembrane protein with six C2 domains which locates to synaptic vesicles. It belongs to the family of ferlin C2 proteins which are implicated in membrane-membrane fusion events.
Otoferlin is expressed in hair cells of the inner ear and in some brain regions. Hair cell ribbon synapses lack the candidate neuronal calcium sensors synaptotagmin 1, synaptotagmin 2 and synaptotagmin 3 and other proteins typical for conventional synapses like synaptophysin.
By analogy of Ca2+ binding, SNARE-binding and because of some aspects of the physiological phenotype of otoferlin-deficient hair cells, otoferlin is suggested to take the role of a Ca2+ sensor of exocytosis in hair cells. Mutations in the otoferlin gene are associated with the recessive prelingual deafness DFNB9.