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Congenital immunodeficiency with a regulatory defect in MHC class II gene expression lacks a specific HLA-DR promoter binding protein, RF-X.

Abstract：

:The expression of MHC class II genes is tightly regulated. One form of congenital severe combined immunodeficiency (SCID) is characterized by a regulatory defect that precludes expression of HLA class II genes. B lymphocyte cell lines from such SCID patients provide a tool for identifying putative regulatory proteins that bind to class II gene promoters. We have identified three proteins binding to specific segments of the HLA-DRA promoter, two of which interact to form the predominant DNA-protein complex observed. One of these proteins, defined as an X box binding protein (RF-X), is specifically missing in cells from class II deficient SCID patients. We propose that the molecular defect in this congenital HLA class II regulatory deficiency is a lack of RF-X and that this factor plays an important role in the normal regulation of MHC class II gene expression.

journal_name

Cell

journal_title

Cell

authors

Reith W,Satola S,Sanchez CH,Amaldi I,Lisowska-Grospierre B,Griscelli C,Hadam MR,Mach B

doi

10.1016/s0092-8674(88)90389-3

subject

Has Abstract

pub_date

1988-06-17 00:00:00

pages

897-906

issue

6

eissn

0092-8674

issn

1097-4172

pii

S0092-8674(88)90389-3

journal_volume

53

pub_type

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