A thiol proteinase inhibitor, E-64-d, corrects the abnormalities in concanavalin a cap formation and the lysosomal enzyme activity in leucocytes from patients with Chediak-Higashi syndrome by reversing the down-regulated protein kinase C activity

Clinical and Experimental Immunology

Volumn 125, Issue 2, 2001, Pages 283-290

  Cui, S H ^a   Tanabe, F ^b   Terunuma, H ^a   Iwatani, Y ^a   Nunoi, H ^c   Agematsu, K ^d   Komiyama, A ^d   Nomura, A ^e   Hara, T ^e   Onodera, T ^f   Iwata, T ^g   Ito, M ^a  

^a UNIVERSITY OF YAMANASHI   (Japan)

^b Japan   (Japan)

^c KUMAMOTO UNIV SCHOOL OF MEDICINE   (Japan)

^d SHINSHU UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^e KYUSHU UNIVERSITY   (Japan)

^f Suita Municipal Hospital   (Japan)

^g UNIVERSITY OF TOKYO   (Japan)

Author keywords

Calpain; Ceramide; Chediak Higashi syndrome; Lysosome; Protein kinase C

Indexed keywords

ALOXISTATIN; CALPAIN; CATHEPSIN G; CERAMIDE; CHELERYTHRINE; CONCANAVALIN A; CYSTEINE PROTEINASE INHIBITOR; ELASTASE; PROTEIN KINASE C INHIBITOR; SPHINGOMYELIN;

ARTICLE; CAPPING PHENOMENON; CASE REPORT; CELL LINE; CHEDIAK HIGASHI SYNDROME; CONTROLLED STUDY; ENZYME ACTIVITY; EPSTEIN BARR VIRUS; FEMALE; FIBROBLAST; HUMAN; HUMAN CELL; LEUKOCYTE; LYSOSOME; MALE; MONOCYTE; NATURAL KILLER CELL; NEUTROPHIL; PRIORITY JOURNAL; PROTEIN DEGRADATION; SCHOOL CHILD;

CALPAIN; CATHEPSINS; CELL LINE, TRANSFORMED; CERAMIDES; CHEDIAK-HIGASHI SYNDROME; CHILD; CONCANAVALIN A; CYSTEINE PROTEINASE INHIBITORS; DOWN-REGULATION; DRUG ANTAGONISM; FEMALE; HUMANS; LEUCINE; LEUKOCYTES, MONONUCLEAR; LYSOSOMES; MALE; PANCREATIC ELASTASE; PROTEIN KINASE C; RECEPTOR AGGREGATION; SERINE ENDOPEPTIDASES; SPHINGOMYELIN PHOSPHODIESTERASE;

EID: 0034881566     PISSN: 00099104     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1365-2249.2001.01598.x     Document Type: Article

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