Home LiteratureArticle Details

PMID: 10583958 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

An essential role for BLNK in human B cell development.

Science (New York, N.Y.) ·Vol. 286 ·No. 5446 ·1999-12-03 ·Pages 1954-7

Minegishi Y, Rohrer J, Coustan-Smith E, Lederman HM, Pappu R, Campana D, Chan AC, Conley ME

Abstract

The signal transduction events that control the progenitor B cell (pro-B cell) to precursor B cell (pre-B cell) transition have not been well delineated. In evaluating patients with absent B cells, a male with a homozygous splice defect in the cytoplasmic adapter protein BLNK (B cell linker protein) was identified. Although this patient had normal numbers of pro-B cells, he had no pre-B cells or mature B cells, indicating that BLNK plays a critical role in orchestrating the pro-B cell to pre-B cell transition. The immune system and overall growth and development were otherwise normal in this patient, suggesting that BLNK function is highly specific.

MeSH Terms

Adaptor Proteins, Signal Transducing Adult Agammaglobulinaemia Tyrosine Kinase Agammaglobulinemia/genetics,immunology Antigens, CD19/analysis Antigens, CD34/analysis B-Lymphocytes/cytology Bone Marrow Cells/cytology Carrier Proteins/genetics,physiology Cell Differentiation Chromosome Mapping Gene Rearrangement, B-Lymphocyte, Heavy Chain Hematopoietic Stem Cells/cytology Humans Male Molecular Sequence Data Phosphoproteins Point Mutation Protein-Tyrosine Kinases/genetics,physiology Reverse Transcriptase Polymerase Chain Reaction Signal Transduction

Chemicals

Adaptor Proteins, Signal Transducing Antigens, CD19 Antigens, CD34 B cell linker protein Carrier Proteins Phosphoproteins Protein-Tyrosine Kinases Agammaglobulinaemia Tyrosine Kinase

Authors & Affiliations

8 authors, click to expand affiliations / ORCID

Minegishi Y

Department of Immunology, Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.

Rohrer J

Coustan-Smith E

Lederman H M

Pappu R

Campana D

Chan A C

Conley M E

Article Info

Journal

Science (New York, N.Y.)

Abbr.

Science

ISSN

0036-8075

DOI

10.1126/science.286.5446.1954

Published

1999-12-03

Pages

1954-7

Language

English

Region

United States

NLM ID

0404511

Subset

Grants

NIAID NIH HHS · AI25129 · United States

NIAID NIH HHS · AI42787 · United States

NCI NIH HHS · CA71516 · United States

Databases

GENBANK

AF180740, AF180741, AF180742, AF180743, AF180744, AF180745, AF180746, AF180747, AF180748, AF180749, AF180750, AF180751, AF180752, AF180753, AF180754, AF180755, AF180756

External Links

PubMed source DOI: 10.1126/science.286.5446.1954 View journal details

Analysis Services

An essential role for BLNK in human B cell development.

Contact

WeChat Official Account