COCH / Cochlin ( isoform short ) Protein

COCH / Cochlin ( isoform short ) Protein

COCH / Cochlin ( isoform short ) Protein Product Information

• Synonym: COCH, UNQ257/PRO294, COCH-5B2, COCH5B2, DFNA9
• Protein Construction: A DNA sequence encoding the mature form of human COCH (NP_001128530.1) (Glu 25-Gln 550) was expressed, with a polyhistidine tag at the N-terminus
• Source: Human
• Expression Host: Human Cells

COCH / Cochlin ( isoform short ) Protein QC Testing

• Purity: > 92 % as determined by SDS-PAGE
• Endotoxin: < 1.0 EU per μg of the protein as determined by the LAL method.
• Stability: Samples are stable for up to twelve months from date of receipt at -70℃
• Predicted N terminal: His
• Molecular Mass: The recombinant full length of human COCH consists of 542 amino acids and has a calculated molecular mass of 59.4 kDa. In SDS-PAGE under reducing conditions, the apparent molecular mass of rhCOCH is approximately 18 kDa, corresponding to the alternative splicing short isoform, whivch lacks two VWFA domains
• Formulation: Lyophilized from sterile PBS , pH 7.4
• SDS-PAGE: COCH protein

COCH / Cochlin ( isoform short ) Protein Usage Guide

• Storage: Store it under sterile conditions at -70℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.

COCH / Cochlin Protein Description

Cochlin, also known as COCH-5B2 and COCH, is a secreted protein which contains one LCCL domain and two VWFA domains. Cochlin / COCH is an abundant inner ear protein expressed as multiple isoforms. Its function is also unknown, but it is suspected to be an extracellular matrix component. Cochlin / COCH and type I I collagen are major constituents of the inner ear extracellular matrix, and COCH constitutes 70% of non-collagenous protein in the inner ear and the expression is highly specific. The co-localization of cochlin and type I I collagen in the fibrillar substance in the subepithelial area indicate that cochlin may play a role in the structural homeostasis of the vestibule acting in concert with the fibrillar type I I collagen bundles. Human Cochlin contains one LCCL and two VWFA domains (VWFA1 and VWFA2 ), and exists as several isoforms which is regulated, at least in part, by alternative splicing at the transcriptional level. The predominant isoforms are the 63 kDa full length COCH, two 40 and 46 kDa isoforms that lack the LCCL domain, and a 16 kDa isoform that lacks two VWFA domains. Defects in COCH are the cause of deafness autosomal dominant type 9 (DFNA9) which is a form of sensorineural hearing loss. DFNA9 is characterized by onset in the fourth or fifth decade of life and initially involves the high frequencies. Defects in COCH may contribute to Meniere disease which is an autosomal dominant disorder characterized by hearing loss associated with episodic vertigo

References

1. Bhattacharya, SK. 2006, Exp Eye Res  82 (3):355-6.
2. Kommareddi,PK. et al., 2007, J Assoc Res Otolaryngol. 8 (4):435-46.
3. Shindo,S.et al., 2008, Neurosci Lett. 444 (2): 148-52.
4. Mizuta,K. Neurosci Lett 2008, 434 (1):104-7.
5. Sekine,K. et al., 2010, Audiol Neurootol. 15 (2):88-96.