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Anti-p47-phox (P366) Antibody

品牌：Antibodies

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规格：50µl

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Anti-p47-phox (P366) Antibody

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Target

Function:	Most upstream protease of the activation cascade of caspases responsible for the TNFRSF6/FAS mediated and TNFRSF1A induced cell death. Binding to the adapter molecule FADD recruits it to either receptor. The resulting aggregate called death-inducing signaling complex (DISC) performs CASP8 proteolytic activation. The active dimeric enzyme is then liberated from the DISC and free to activate downstream apoptotic proteases. Proteolytic fragments of the N-terminal propeptide (termed CAP3, CAP5 and CAP6) are likely retained in the DISC. Cleaves and activates CASP3, CASP4, CASP6, CASP7, CASP9 and CASP10. May participate in the GZMB apoptotic pathways. Cleaves ADPRT. Hydrolyzes the small-molecule substrate, Ac-Asp-Glu-Val-Asp-\|-AMC. Likely target for the cowpox virus CRMA death inhibitory protein. Isoform 5, isoform 6, isoform 7 and isoform 8 lack the catalytic site and may interfere with the pro-apoptotic activity of the complex.
Tissue Specificity:	Isoform 1, isoform 5 and isoform 7 are expressed in a wide variety of tissues. Highest expression in peripheral blood leukocytes, spleen, thymus and liver. Barely detectable in brain, testis and skeletal muscle.
Involvement in Disease:	Caspase-8 deficiency: Disorder resembling autoimmune lymphoproliferative syndrome (ALPS). It is characterized by lymphadenopathy, splenomegaly, and defective CD95-induced apoptosis of peripheral blood lymphocytes (PBLs). It leads to defects in activation of T-lymphocytes, B-lymphocytes, and natural killer cells leading to immunodeficiency characterized by recurrent sinopulmonary and herpes simplex virus infections and poor responses to immunization.
Sequence Similarities:	Belongs to the peptidase C14A family.
Post-Translational Modification:	Generation of the subunits requires association with the death-inducing signaling complex (DISC), whereas additional processing is likely due to the autocatalytic activity of the activated protease. GZMB and CASP10 can be involved in these processing events.
Cellular locations:	Cytoplasm.
Database Links:	Entrez Gene: 841 Human Entrez Gene: 12370 Mouse Entrez Gene: 64044 Rat Omim: 601763 Human SwissProt: Q14790 Human SwissProt: O89110 Mouse SwissProt: Q9JHX4 Rat Unigene: 599762 Human Unigene: 336851 Mouse Unigene: 54474 Rat
Synonyms:	ALPS2B Antibody Amyotrophic lateral sclerosis 2 chromosomal region candidate gene 12 protein Antibody Apoptotic cysteine protease Antibody Apoptotic protease Mch-5 Antibody Apoptotic protease Mch5 Antibody CAP4 Antibody CASP-8 Antibody CASP8 Antibody CASP8_HUMAN Antibody Caspase 8 Antibody Caspase 8 apoptosis related cysteine peptidase Antibody Caspase-8 Antibody Caspase-8 subunit p10 Antibody CED 3 Antibody FADD Like ICE Antibody FADD-homologous ICE/CED-3-like protease Antibody FADD-like ICE Antibody FLICE Antibody FLJ17672 Antibody ICE-like apoptotic protease 5 Antibody MACH Antibody MACH alpha 1/2/3 protein Antibody MACH beta 1/2/3/4 protein Antibody MCH5 Antibody MGC78473 Antibody MORT1 associated ced 3 homolog Antibody MORT1-associated CED-3 homolog Antibody OTTHUMP00000163717 Antibody OTTHUMP00000163720 Antibody OTTHUMP00000163724 Antibody OTTHUMP00000163725 Antibody OTTHUMP00000165062 Antibody OTTHUMP00000165063 Antibody OTTHUMP00000165064 Antibody OTTHUMP00000206552 Antibody OTTHUMP00000206582 Antibody
Information:	Target information shown above is from the UniProt Consortium.