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Assembly and function of the two ABC transporter proteins encoded in the human major histocompatibility complex | | A Kelly
; S H Powis
; L A Kerr
; I Mockridge
; T Elliott
; J Bastin
; B Uchanska-Ziegler
; A Ziegler
; J Trowsdale
; A Townsend
; | | Date: |
13 Feb 1992 | | Journal: | Nature, 355 (6361), 641-4 | | Abstract: | Presentation of cytoplasmic antigens to class I-restricted cytotoxic T cells implied the existence of a specialized peptide transporter. For most class I heavy chains, association with peptides of the appropriate length is required for stable assembly with beta 2-microglobulin. Mutant cells RMA-S and .174/T2 neither assemble stable class I molecules nor present intracellular antigens, and we have suggested that they have lost a function required for the transport of short peptides from the cytosol to the endoplasmic reticulum. The genetic defect in .174 has been localized to a large deletion in the class II region of the major histocompatibility complex, within which two genes (RING4 and RING11) have been identified that code for ’ABC’ (ATP-binding cassette) transporters. We report here that the protein products of these two genes assemble to form a complex. Defects in either protein result in the formation of unstable class I molecules and loss of presentation of intracellular antigens. The molecular defect in a new mutant, BM36.1, is shown to be in the ATP-binding domain of the RING11/PSF2 protein. This is in contrast to the mutant .134, which lacks the RING4/PSF1 protein. | | Source: | PubMed, pmid1538751 doi: 10.1038/355641a0 | | Services: | Forum | Review | Favorites | |
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