Recurrent infections and delayed separation of the umbilical cord in an infant with abnormal phagocytic cell locomotion and oxidative response during particle phagocytosis

Abramson, Jon S.; Mills, Elaine L.; Sawyer, Mary Kirk; Regelmann, Warren E.; Nelson, John D.; Quie, Paul G.

doi:10.1016/s0022-3476(81)80011-x

Cited by 51 publications

(15 citation statements)

References 16 publications

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“…Studies of patient cell secretion in response to stimulation with CFs or PMA and "regurgitation" during phagocytosis of zymosan particles are summarized in Table III Table IV demonstrate considerable heterogenity of phagocytosis by GP138-deficient PMNs with respect to the test particle employed. Findings of diminished uptake by patient cells of radiolabeled staphylococcal organisms or zymosan confirmed previous findings for these test particles (6). Their uptake of Oil-Red-0-paraffin emulsions selectively opsonized with C3-derived ligands was also significantly (P < 0.01) diminished compared with healthy adult or maternal controls, but their uptake of IgG opsonized Oil-Red-0 emulsions was only slightly diminished compared with that of control suspensions (P > 0.05).…”

Section: Sds-page Results Of Sds-pagesupporting

confidence: 86%

“…Investigations described in this report have identified a total deficiency of PMN and monocyte Mol and LFA1 in a female patient with a similar clinical syndrome (6). These studies were designed to explore the role of these glycoproteins in allowing normal PMNs to interact with experimental or biologic substrates, phagocytizable test particles, or the surfaces of other cell types, and to determine their topographical and/or functional relationships to selected PMN membrane receptors.…”

Section: Introductionmentioning

confidence: 96%

See 1 more Smart Citation

Abnormalities of polymorphonuclear leukocyte function associated with a heritable deficiency of high molecular weight surface glycoproteins (GP138): common relationship to diminished cell adherence.

Anderson¹,

Schmalstieg²,

Arnaout³

et al. 1984

J. Clin. Invest.

257

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Section: Sds-page Results Of Sds-pagesupporting

confidence: 86%

Section: Introductionmentioning

confidence: 96%

Abnormalities of polymorphonuclear leukocyte function associated with a heritable deficiency of high molecular weight surface glycoproteins (GP138): common relationship to diminished cell adherence.

Anderson¹,

Schmalstieg²,

Arnaout³

et al. 1984

J. Clin. Invest.

257

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“…This LFA-l variation contrasts with the inherited immunodeficiency syndrome involving the partial or complete deficiency of the Mac-1, LFA-I family of molecules (15)(16)(17)(18)(19)(20)(21)(22)(23). Those patients synthesize normal a-chain precursor molecules but apparently don't synthesize normal ,-chains and as a consequence few dimers are formed (24,25).…”

Section: Resultsmentioning

confidence: 99%

“…5, lanes 6 and 14). Since an immunodeficiency syndrome involving the absence ofthe three related molecules constituting the LFA-1 family has been described (15)(16)(17)(18)(19)(20)(21)(22)(23), the cells from the two individuals who did not react with serum E27 were reexamined to determine if they expressed LFA-l molecules on their cell surfaces. Both LFA-l a-and LFA-1 (3-chains could be immunoprecipitated from cells of both individuals by specific MAb (Fig.…”

Section: Resultsmentioning

confidence: 99%

Polymorphism of lymphocyte function-associated antigen-1 demonstrated by a lupus patient's alloantiserum.

Pischel¹,

Marlin²,

Springer³

et al. 1987

J. Clin. Invest.

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We have found a human serum, E27, obtained from a multiply transfused patient with systemic lupus erythematosus, which immunoprecipitates the lymphocyte function associated antigen-1 (LFA-1). The immunoprecipitated molecules were identified as the LFA-1 alpha and beta chains by their comigration on SDS-PAGE, two-dimensional SDS-PAGE, and by sequential clearance experiments. Serum E27 did not immunoprecipitate LFA-1 from autologous cells, though LFA-1 molecules were present. In contrast, serum E27 immunoprecipitated LFA-1 from most but not all normal donor lymphocytes. Thus, serum E27 defines two serological phenotypes of LFA-1. 95% of normal individuals tested exhibited the LFA-1 phenotype precipitated by serum E27. Serum E27 appears to be directed at determinants of the LFA-1 a-chain and not the E-chain since it immunoprecipitated LFA-1 molecules but not the Mac-i molecules. Additional evidence for the alpha chain specificity was provided by immunoprecipitation of mouse-human heterohybridoma cells. LFA-1 was immunoprecipitated by serum E27 from mouse-human heterohybridoma cells expressing the human a-chain, not from a hybrid cell line expressing the human f-chain. Together these findings demonstrate an antigenic polymorphism of the human LFA-1 a-chain molecule.

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“…As mentioned earlier, at least 13 individuals from 1 1 families, including our sister and brother, have recently been described with a neutrophil adhesion disorder (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12), most likely inherited in an autosomal recessive fashion (1,12). The use of SDS-PAGE for studies of leukocyte surface glycoproteins deficient in these patients has contributed to considerable confusion concerning the identity of the disease.…”

Section: Discussionmentioning

confidence: 99%

Neutrophil Adhesion Abnormality with Deficient Surface Membrane Proteins (gp 110 and p 98): The Effect of their Antibodies on the Function of Normal Neutrophils

et al. 1986

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ABSTRACT. A sister and brother previously described with neutrophil adhesion defects and a lack of two neutrophi1 membrane proteins, glycoprotein with a molecular weight of 110 K and 115 K, were further studied. Rabbit polyclonal IgG antibodies were raised against neutrophil membrane proteins of approximately 110 K from normal individuals, and absorbed with the siblings' neutrophil membrane proteins. The antibodies thus absorbed reacted with two normal neutrophil membrane proteins, a 110 K glycoprotein and a 9 8 K protein, which were totally deficient in the siblings' neutrophils. The polyclonal antibodies were also reactive with a 9 5 K membrane protein of normal lymphocytes. The protein was missing in the siblings' lymphocytes. Three membrane proteins, Mac-1, LFA-1, and p 150, 9 5 each consisting of a specific a-subunit and a P-subunit common to them, have been reported to be deficient in neutrophils from several patients with a neutrophil adhesion disease. Using monoclonal antibodies to Mac-la, LFA-la, and to the P-subunit, it was deduced that the siblings' neutrophils lacked the above three membrane proteins. The disease in the siblings is thus identical with that previously described. Sodium dodecvl sulfate polyacryla~ide gel ~l e~t r o~h o r e s i s of immunopr~cipitates, formed bv reacting labeled normal neutro~hil membrane -proteins with our polyclonal antibodies, revealed three bands, almost identical with that of immunoprecipitates formed with the anti-P-subunit monoclonal antibody. This finding indicates that the polyclonal antibodies reacted mainly with the P-subunit of the membrane proteins. The presence of the polyclonal antibodies exerted a strong inhibitory effect upon the adhesion of normal neutrophils, an intermediate effect on the chemotaxis, and to a much lesser extent on the phagocytosis. (Pediatr Res 20: 361-366,1986) Abbreviations gp 110, glycoprotein with a molecular weight of 110 K gp 115, glycoprotein with a molecular weight of 115 K p 98, protein with a molecular weight of 9 8 K PBS, phosphate-buffered saline SDS-PAGE, sodium dodecyl sulfate-polyacrylamide gel electrophoresisWe have described a sister and brother who suffered from recurrent bacterial infections and whose neutrophils exhibited impaired adhesion, chemotaxis, and phagocytosis (I, 2). Their neutrophils lacked two membrane glycoproteins: one with a molecular weight of 110 K (gp 110) present on the cell surface, and the other with a molecular weight of 1 15 K (gp 1 15) possibly situated in the intracellular apparatuses. Their parents, both healthy, showed a reduced rate of neutrophil adhesion and reduced levels of the two glycoproteins. The disease was thus inherited in an autosomal recessive fashion. Scanning electron microscopy of the neutrophils from the brother attached on a glass surface revealed a considerable reduction of the pseudopods. On the other hand, his neutrophils in suspension were morphologically normal, a finding indicating that the cytoskeleton of the neutrophils was functionally intact (2). Several rec...

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Recurrent infections and delayed separation of the umbilical cord in an infant with abnormal phagocytic cell locomotion and oxidative response during particle phagocytosis

Cited by 51 publications

References 16 publications

Abnormalities of polymorphonuclear leukocyte function associated with a heritable deficiency of high molecular weight surface glycoproteins (GP138): common relationship to diminished cell adherence.

Abnormalities of polymorphonuclear leukocyte function associated with a heritable deficiency of high molecular weight surface glycoproteins (GP138): common relationship to diminished cell adherence.

Polymorphism of lymphocyte function-associated antigen-1 demonstrated by a lupus patient's alloantiserum.

Neutrophil Adhesion Abnormality with Deficient Surface Membrane Proteins (gp 110 and p 98): The Effect of their Antibodies on the Function of Normal Neutrophils

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