TFIIH, native complex human

TFIIH is a multicomponent basal transcription factor complex. Nine subunits have been identified within the TFIIH holoenzyme complex. Various enzymatic activities, including DNA repair, helicase, and cyclin-dependent kinase activities, have been reported. The XPB, p62, p52, p44, and p34 subunits are thought to constitute the "core" of the TFIIH transcription machinery. Although the p44 and p34 subunits have no defined enzymatic activity, their zinc finger structures suggest that they may be DNA-binding proteins that might mediate interactions with soluble transcription factors. The cdk-activating kinase (CAK) subcomplex, comprising subunits Cdk7, cyclin H, amd MAT1, phosphorylate several cyclin-dependent kinases (cdks), as well as the carboxy-terminal domain of pol II. Several inherited human disorders such as Xeroderma pigmentosum (XP), Cockayne syndrome (CS) and trichothiodystrophy (TTD) are associated with mutations in TFIIH subunits.

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