Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia

Ornella Parolini; Satoshi Tsukada; Douglas C. Saffran; David J. Rawlings; R.Cutler Allen; Ivana Klisak; Robert S. Sparkes; Hiromi Kubagawa; Thuluvancheri Mohandas; Shirley Quan; John W. Belmont; Max D. Cooper; Mary Ellen Conley; Owen N. Witte

doi:10.1016/0092-8674(93)90667-F

Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia

Ornella Parolini, Satoshi Tsukada, Douglas C. Saffran, David J. Rawlings, R.Cutler Allen, Ivana Klisak, Robert S. Sparkes, Hiromi Kubagawa, Thuluvancheri Mohandas, Shirley Quan, John W. Belmont, Max D. Cooper, Mary Ellen Conley, Owen N. Witte

Risultato della ricerca: Contributo in rivista › Articolo in rivista

1057 Citazioni (Scopus)

Abstract

We describe a novel cytoplasmic tyrosine kinase, termed BPK (B cell progenitor kinase), which is expressed in all stages of the B lineage and in myeloid cells. BPK has classic SH1, SH2, and SH3 domains, but lacks myristylation signals and a regulatory phosphorylation site corresponding to tyrosine 527 of c-src. BPK has a long, basic amino-terminal region upstream of the SH3 domain. BPK was evaluated as a candidate for human X-linked agammaglobulinemia (XLA), an inherited immunodeficiency characterized by a severe deficit of B and plasma cells and profound hypogammaglobulinemia. BPK mapped to within 100 kb of a probe defining the polymorphism most closely linked to XLA at DXS178. Reduction in or the absence of BPK mRNA, protein expression, and kinase activity was observed in XLA pre-B and B cell lines. BPK is likely the XLA gene and functions in pathways critical to B cell expansion.

Lingua originale	English
pagine (da-a)	279-290
Numero di pagine	12
Rivista	Cell
Volume	72
DOI	https://doi.org/10.1016/0092-8674(93)90667-F
Stato di pubblicazione	Pubblicato - 1993

Keywords

Agammaglobulinemia
Amino Acid Sequence
Animals
B-Lymphocytes
Blotting, Northern
Blotting, Southern
Cell Line
Chromosome Mapping
Cloning, Molecular
Cosmids
Cytoplasm
DNA
Heterozygote Detection
Humans
Hybrid Cells
Mice
Molecular Sequence Data
Protein-Tyrosine Kinases
RNA, Messenger
Sequence Homology, Amino Acid
Transcription, Genetic
Tumor Cells, Cultured
X Chromosome

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Parolini, O., Tsukada, S., Saffran, D. C., Rawlings, D. J., Allen, R. C., Klisak, I., Sparkes, R. S., Kubagawa, H., Mohandas, T., Quan, S., Belmont, J. W., Cooper, M. D., Conley, M. E., & Witte, O. N. (1993). Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia. Cell, 72, 279-290. https://doi.org/10.1016/0092-8674(93)90667-F

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title = "Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia",

abstract = "We describe a novel cytoplasmic tyrosine kinase, termed BPK (B cell progenitor kinase), which is expressed in all stages of the B lineage and in myeloid cells. BPK has classic SH1, SH2, and SH3 domains, but lacks myristylation signals and a regulatory phosphorylation site corresponding to tyrosine 527 of c-src. BPK has a long, basic amino-terminal region upstream of the SH3 domain. BPK was evaluated as a candidate for human X-linked agammaglobulinemia (XLA), an inherited immunodeficiency characterized by a severe deficit of B and plasma cells and profound hypogammaglobulinemia. BPK mapped to within 100 kb of a probe defining the polymorphism most closely linked to XLA at DXS178. Reduction in or the absence of BPK mRNA, protein expression, and kinase activity was observed in XLA pre-B and B cell lines. BPK is likely the XLA gene and functions in pathways critical to B cell expansion.",

keywords = "Agammaglobulinemia, Amino Acid Sequence, Animals, B-Lymphocytes, Blotting, Northern, Blotting, Southern, Cell Line, Chromosome Mapping, Cloning, Molecular, Cosmids, Cytoplasm, DNA, Heterozygote Detection, Humans, Hybrid Cells, Mice, Molecular Sequence Data, Protein-Tyrosine Kinases, RNA, Messenger, Sequence Homology, Amino Acid, Transcription, Genetic, Tumor Cells, Cultured, X Chromosome, Agammaglobulinemia, Amino Acid Sequence, Animals, B-Lymphocytes, Blotting, Northern, Blotting, Southern, Cell Line, Chromosome Mapping, Cloning, Molecular, Cosmids, Cytoplasm, DNA, Heterozygote Detection, Humans, Hybrid Cells, Mice, Molecular Sequence Data, Protein-Tyrosine Kinases, RNA, Messenger, Sequence Homology, Amino Acid, Transcription, Genetic, Tumor Cells, Cultured, X Chromosome",

author = "Ornella Parolini and Satoshi Tsukada and Saffran, {Douglas C.} and Rawlings, {David J.} and R.Cutler Allen and Ivana Klisak and Sparkes, {Robert S.} and Hiromi Kubagawa and Thuluvancheri Mohandas and Shirley Quan and Belmont, {John W.} and Cooper, {Max D.} and Conley, {Mary Ellen} and Witte, {Owen N.}",

year = "1993",

doi = "10.1016/0092-8674(93)90667-F",

language = "English",

volume = "72",

pages = "279--290",

journal = "Cell",

issn = "0092-8674",

publisher = "Cell Press:1100 Massachusetts Avenue:Cambridge, MA 02138:(617)661-7057, EMAIL: subs@cell.com, INTERNET: http://www.cellpress.com, Fax: (617)397-2850",

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TY - JOUR

T1 - Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia

AU - Parolini, Ornella

AU - Tsukada, Satoshi

AU - Saffran, Douglas C.

AU - Rawlings, David J.

AU - Allen, R.Cutler

AU - Klisak, Ivana

AU - Sparkes, Robert S.

AU - Kubagawa, Hiromi

AU - Mohandas, Thuluvancheri

AU - Quan, Shirley

AU - Belmont, John W.

AU - Cooper, Max D.

AU - Conley, Mary Ellen

AU - Witte, Owen N.

PY - 1993

Y1 - 1993

N2 - We describe a novel cytoplasmic tyrosine kinase, termed BPK (B cell progenitor kinase), which is expressed in all stages of the B lineage and in myeloid cells. BPK has classic SH1, SH2, and SH3 domains, but lacks myristylation signals and a regulatory phosphorylation site corresponding to tyrosine 527 of c-src. BPK has a long, basic amino-terminal region upstream of the SH3 domain. BPK was evaluated as a candidate for human X-linked agammaglobulinemia (XLA), an inherited immunodeficiency characterized by a severe deficit of B and plasma cells and profound hypogammaglobulinemia. BPK mapped to within 100 kb of a probe defining the polymorphism most closely linked to XLA at DXS178. Reduction in or the absence of BPK mRNA, protein expression, and kinase activity was observed in XLA pre-B and B cell lines. BPK is likely the XLA gene and functions in pathways critical to B cell expansion.

AB - We describe a novel cytoplasmic tyrosine kinase, termed BPK (B cell progenitor kinase), which is expressed in all stages of the B lineage and in myeloid cells. BPK has classic SH1, SH2, and SH3 domains, but lacks myristylation signals and a regulatory phosphorylation site corresponding to tyrosine 527 of c-src. BPK has a long, basic amino-terminal region upstream of the SH3 domain. BPK was evaluated as a candidate for human X-linked agammaglobulinemia (XLA), an inherited immunodeficiency characterized by a severe deficit of B and plasma cells and profound hypogammaglobulinemia. BPK mapped to within 100 kb of a probe defining the polymorphism most closely linked to XLA at DXS178. Reduction in or the absence of BPK mRNA, protein expression, and kinase activity was observed in XLA pre-B and B cell lines. BPK is likely the XLA gene and functions in pathways critical to B cell expansion.

KW - Agammaglobulinemia

KW - Amino Acid Sequence

KW - Animals

KW - B-Lymphocytes

KW - Blotting, Northern

KW - Blotting, Southern

KW - Cell Line

KW - Chromosome Mapping

KW - Cloning, Molecular

KW - Cosmids

KW - Cytoplasm

KW - DNA

KW - Heterozygote Detection

KW - Humans

KW - Hybrid Cells

KW - Mice

KW - Molecular Sequence Data

KW - Protein-Tyrosine Kinases

KW - RNA, Messenger

KW - Sequence Homology, Amino Acid

KW - Transcription, Genetic

KW - Tumor Cells, Cultured

KW - X Chromosome

KW - Agammaglobulinemia

KW - Amino Acid Sequence

KW - Animals

KW - B-Lymphocytes

KW - Blotting, Northern

KW - Blotting, Southern

KW - Cell Line

KW - Chromosome Mapping

KW - Cloning, Molecular

KW - Cosmids

KW - Cytoplasm

KW - DNA

KW - Heterozygote Detection

KW - Humans

KW - Hybrid Cells

KW - Mice

KW - Molecular Sequence Data

KW - Protein-Tyrosine Kinases

KW - RNA, Messenger

KW - Sequence Homology, Amino Acid

KW - Transcription, Genetic

KW - Tumor Cells, Cultured

KW - X Chromosome

UR - http://hdl.handle.net/10807/92526

U2 - 10.1016/0092-8674(93)90667-F

DO - 10.1016/0092-8674(93)90667-F

M3 - Article

VL - 72

SP - 279

EP - 290

JO - Cell

JF - Cell

SN - 0092-8674

ER -

Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia

Abstract

Keywords

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