GM17557
LCL from B-Lymphocyte
Description:
IMMUNODEFICIENCY WITH HYPER-IGM, TYPE 2
Repository
|
NIGMS Human Genetic Cell Repository
|
Subcollection |
Heritable Diseases |
Class |
Disorders of Uncertain Biochemical Etiology |
Biopsy Source
|
Peripheral vein
|
Cell Type
|
B-Lymphocyte
|
Tissue Type
|
Blood
|
Transformant
|
Epstein-Barr Virus
|
Sample Source
|
LCL from B-Lymphocyte
|
Race
|
White
|
Relation to Proband
|
proband
|
Confirmation
|
Clinical summary/Case history
|
Species
|
Homo sapiens
|
Common Name
|
Human
|
Remarks
|
|
IDENTIFICATION OF SPECIES OF ORIGIN |
Species of Origin Confirmed by Nucleoside Phosphorylase,Glucose-6-Phosphate Dehydrogenase, and Lactate Dehydrogenase Isoenzyme Electrophoresis |
|
Remarks |
Line LAZ248; at age 7 patient had recurrent pyogenic infections; IgG<100mg/dl; IgM>50mg/dl (sometimes as high as 200); IgA<50mg/dl; the cells express surface IgM and IgD, but not IgG |
Schwaber JF, Rosen FS, Isotypes of surface immunoglobulin on B lymphocytes from patients with immune deficiency. J Clin Immunol2(1):30-4 1982 |
PubMed ID: 6980225 |
|
Schwaber JF, Lazarus H, Rosen FS, IgM-restricted production of immunoglobulin by lymphoid cell lines from patients with immunodeficiency with hyper IgM (dysgammaglobulinemia). Clin Immunol Immunopathol19(1):91-7 1981 |
PubMed ID: 7011617 |
Split Ratio |
1:3 |
Temperature |
37 C |
Percent CO2 |
5% |
Medium |
Roswell Park Memorial Institute Medium 1640 with 2mM L-glutamine or equivalent |
Serum |
15% fetal bovine serum Not Inactivated |
Substrate |
None specified |
Subcultivation Method |
dilution - add fresh medium |
Supplement |
- |
|
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