Target intelligence / Profile preview

Otolin-1

Target
Otolin-1
Molecular classification
Glycoprotein, Extracellular matrix structural protein, Collagen-like protein, C1q/TNF-related protein family
01

Overview

Otolin-1 is a secreted collagen-like glycoprotein primarily found in the inner ear. It is crucial for the formation and maintenance of otoconia, which are calcium carbonate crystals responsible for proper sensing of balance and spatial orientation. Otolin-1 provides the organic scaffold that supports biomineralization and interacts with other matrix proteins, especially otoconin-90 and cerebellin-1, to modulate the morphology and growth kinetics of calcite crystals comprising otoconia. Its structure includes a signal peptide, an N-terminal cysteine-rich region, a collagen-like domain, and a highly conserved globular C1q-like domain that mediates trimerization and calcium binding. Mutations in the *OTOL1* gene are linked to abnormal otoconia formation and balance disorders, most notably benign paroxysmal positional vertigo (BPPV). Otolin-1 can be detected in blood, where elevated levels correlate with otoconia degradation and vestibular dysfunction, making it a promising biomarker for certain inner ear diseases[1][2][3][4][5][6].

Other names
OtolinOTOL1Otolin proteinCollagen-like protein OTOL1
02

Biological functions

Scaffold for biomineralization in inner earCalcium ion bindingExtracellular matrix structural constituentInteracts with otoconin-90 and cerebellin-1 to form otoconia (organic matrix)
03

Disease associations

Benign paroxysmal positional vertigo (BPPV)Vestibular disorders (balance deficits, peripheral vertigo, vestibular neuronitis)
04

Biomarkers

Blood/serum levels used as a biomarker for otoconia degeneration and BPPV risk/recurrencePotential marker for acute inner ear disorders and age-related vestibular dysfunction

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