Immunodeficiency to the Epstein-Barr virus in the X-linked recessive lymphoproliferative syndrome

Purtilo, David T.; Hutt, Lindsey M.; Bhawan, Jag; Yang, James P. S.; Cassel, Christine K.; Allegra, Salvatore R.; Rosen, Fred S.

doi:10.1016/0090-1229(78)90066-1

Cited by 57 publications

(13 citation statements)

References 22 publications

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“…Virus used for Absorption (Virions x 1 06) FIGURE 4 Analysis of the viral specificity of the nonimmune IgG reactive with HSV-1 and with EBV. Purified nonimmune IgG (90 Mg) was preabsorbed with varying amounts (1.25 X 106 to 1 X 107 virions) of isolated EBV (0 *), HSV-1 (A A) or VSV (U U) prior to being assayed undiluted for residual EBV neutralizing activity in the presence of purified Cl, C4, C2, and C3 (upper panel) or for EBV ELISA reactivity (lower panel).…”

Section: Resultsmentioning

confidence: 99%

“…Next, 2 X 105 CBL were added and incubation continued for 60 min at 37°C in a 5% CO2 humidified incubator. The CBL were then washed with RPMI media, plated in microtiter plates, and pulsed with 2 gCi of [3H]thymidine 4 Absorption of nonimmune IgG and serum with virus. Purified EBV, HSV-1, CMV, or VSV were used to absorb either serum or purified IgG obtained from individuals nonimmune to EBV.…”

Section: Methodsmentioning

confidence: 99%

“…In most cases this disorder is self-limiting, but it can progress to a fatal lymphoproliferative disease in immune-deficient individuals (3,4). In addition, EBV is an oncogenic agent in certain nonhuman primates (5) and is a prime candidate for a human cancer virus (6,7).…”

Section: Introduction Epstein-barr Virus (Ebv)' Infection In Man Usuallymentioning

confidence: 99%

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Neutralization of Epstein-Barr Virus by Nonimmune Human Serum

Nemerow¹,

Jensen²,

Cooper³

1982

J. Clin. Invest.

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A B S T R A C T These studies were carried out to investigate the mechanism of neutralization of purified Epstein-Barr virus (EBV) simplex I (HSV-1) absorbed the EBV ELISA reactivity and EBV-neutralizing activity of nonimmune sera, whereas another member of the herpesvirus group, cytomegalovirus, was inactive in this regard. HSV-1 was quantitatively more efficient than EBV in absorbing reactivity, a finding that indicates that the antibody has a higher affinity for HSV-1 than for EBV. Further absorption studies indicated that the cross-reaction occurred in both directions as EBV also absorbed HSV-1 reactive antibodies as tested in an HSV-1 ELISA. EBV was also less efficient than HSV-1 in absorbing reactivity with HSV-1. A serum lacking detectable antibodies.to both EBV and HSV-1 failed to neutralize EBV. These studies cumulatively indicate that fresh serum from EBV nonimmune individuals neutralizes EBV by the combined action of a previously undescribed cross-reacting antibody apparently elicited by HSV-1 and Cl, C4, C2, and C3 of the classical complement pathway.

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Section: Resultsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Neutralization of Epstein-Barr Virus by Nonimmune Human Serum

Nemerow¹,

Jensen²,

Cooper³

1982

J. Clin. Invest.

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“…In this regard EBV is now thought to be the cause of serious as well as fatal disease in individuals with "X-linked lymphoproliferative syndrome" (34,35). An etiological step in the disease process most likely includes the transformation of peripheral blood leukocytes of these patients by EBV (35). Phosphonoacetic acid has been shown to inhibit the cytopathic effects of HSV in tissue culture (4) and experimental animals (41).…”

Section: Discussionmentioning

confidence: 99%

“…In addition, a new Xlinked recessive lymphoproliferative syndrome has recently emerged which is characterized by subtle immunodeficiency to infectious agents, especially EBV (34). Phenotypes of fatal IM, agammaglobulinemia following IM, American Burkitt's lymphoma, and B-cell sarcoma have been seen by Purtilo et al (34,35) in boys with the syndrome. The results of their recent study suggest that EBV plays an etiological role in these diseases and that defective immunity fails to stop EBV-induced proliferation of B cells in affected males.…”

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confidence: 99%

Effects of Nucleoside Analogs on Epstein-Barr Virus-Induced Transformation of Human Umbilical Cord Leukocytes and Epstein-Barr Virus Expression in Transformed Cells

Henderson

Long

Ribecky

1979

Antimicrob Agents Chemother

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Two methods of assay, measuring (i) stimulation of host cell DNA synthesis by [3H]thymidine incorporation and (ii) morphological transformation in microtiter plates, were employed to determine what effect treatment during infection with adenine arabinoside or 5-iodo-5'-amino-2',5'-dideoxyuridine has on Epstein-Barr virus (EBV) (B95-8)-induced transformation of human umbilical cord leukocytes. It was found that adenine arabinoside inhibited EBV-induced transformation in a dose-dependent manner in both assays, beginning at drug concentrations (<2 ,ug/ml) which had little effect on either spontaneous or phytohemagglutinininduced host cell DNA synthesis. Adenine arabinoside was more effective in inhibiting morphological transformation than EBV-induced host DNA synthesis. Adenine arabinoside treatment was also effective in reducing both EB viral capsid antigen expression and production of biologically active extracellular transforming virus in EBV-transformed cells. In contrast, 5-iodo-5'-amino-2',5'-dideoxyuridine, which inhibited herpes simplex virus replication, had little effect on EBV-induced transformation as measured by either method of assay. However, 5-iodo-5'-amino-2',5'-dideoxyuridine was found to be effective in inhibiting viral capsid antigen expression and production of extracellular transforming virus in EBV-transformed cells.

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