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keratin, type II cuticular Hb3; type II hair keratin Hb3; keratin-83; K83; K2.10 (KRT83 KRTHB3)

Structure: - type-2 hair keratin - heterotetramer of 2 type 1 & 2 type 2 keratins - belongs to the intermediate filament family Expression: - synthesis begins in the cortex 10-15 cell layers above the apex of the dermal papilla & ends abruptly in the middle of the cortex Pathology: - defects in KRTHB3 are a cause of monilethrix

General

basic hair keratin or type II hair keratin

Properties

CONFIGURATION: oligomer SIZE: entity length = 493 aa MW = 54 kD COMPARTMENT: cytoplasm MOTIF: Head {1-111} O-glycosylation site {N-TERMINAL} rod-like {central-region} MOTIF: alpha helix coiled coil Rod {112-418} MOTIF: Coil 1A {112-146} Linker 1 {147-156} Coil 1B {157-257} Linker 12 {258-274} Coil 2 {275-418} glutamate-rich region {C-TERMINAL} MOTIF: glutamate residue (SEVERAL) Ser phosphorylation site (SEVERAL) MOTIF-SEQUENCE: KSP Tail {419-493}

Database Correlations

OMIM correlations MORBIDMAP 602765 UniProt P78385 Pfam PF00038 Entrez Gene 3889

References

  1. Entrez Gene :accession 3889
  2. UniProt :accession P78385
  3. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=KRT83