Fine Structural Studies of Leukocytes from Patients and Heterozygotes with the Chediak-Higashi Syndrome
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چکیده
منابع مشابه
Fine structural studies of leukocytes from patients and heterozygotes with the Chediak-Higashi syndrome.
T HE CHEDIAK-HIGASHI SYNDROME (CR5) is characterized by large abnormal cytoplasmic organelles in cells including leukocytes which contain lysosome-like structures. This rare disease is inherited as an autosomal recessive disorder, and the clinical features include partial oculocutaneous albinism, frequent and severe pyogenic infections, and the development of a lymphoma-like ( accelerated ) pha...
متن کاملThe Chediak-Higashi syndrome: cytoplasmic sequestration in circulating leukocytes.
By JAMES C. WHITE A RARE FAMILIAL DISORDER characterized cytologically by enormous particles in all varieties of leukocytes has been reported from various parts of the world and is known in this country as the Chediak-Higashi (C.H.) syndrome.1-4 The variable clinical manifestations of patients with this disease have included defective pigmentation, neuropathies, lymphadenopathy, hepatosplenomeg...
متن کاملpatient with Chediak-Higashi syndrome
Accepted for publication 8 November 1994 Abstract Aims-To determine whether neutrophil elastase and cathepsin G are expressed, at transcriptional or translational levels, in the bone marrow from a patient with Chediak-Higashi syndrome. Methods-Blood neutrophils were isolated from three patients with Chediak-Higashi disease and bone marrow was collected from one. Cell lysates were analysed for n...
متن کاملChediak-Higashi Syndrome
Impaired vision Photophobia Albinism of the OCA2 type, giving a lighter complexion than unaffected family members [6] Silvery sheen to hair which may be fair in colour Frequent infections (skin, mucous membranes, respiratory) Epilepsy Mental retardation Enlarged liver and spleen Jaundice Ataxia causing incoordination and a typical ataxic gait Tremor Epilepsy Peripheral neuropathy causing motor ...
متن کاملTubulin tyrosinolation in human polymorphonuclear leukocytes: studies in normal subjects and in patients with the Chediak-Higashi syndrome
We have recently reported a specific dose-dependent stimulation of posttranslational incorporation of tyrosine into tubulin alpha-chains of rabbit peritoneal leukocytes as induced by the synthetic peptide chemoattractant formyl-methionyl-leucyl-phenylalanine (FMLP). The present study reports a similar, specific stimulation of tubulin tyrosinolation in human polymorphonuclear leukocytes (PMN). W...
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ژورنال
عنوان ژورنال: Blood
سال: 1969
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood.v33.4.527.527