Medical

CASP10

CASP10

A gene on chromosome 2q33-q34 that encodes a protein belonging to the cysteine-aspartic acid protease (caspase) family which, once activated by proteolytic processing, plays a central role in the execution-phase of cell apoptosis, as well as various stages of embryological development. CASP10 is an initiator-type caspase which is activated by, and interacts with, upstream adaptor molecules through CARD and DED protein–protein interaction domains. It cleaves and activates caspase-3, -4, -6, -7, -8 and -9, is recruited to Fas- and TNFR-1 receptors in a FADD-dependent manner, and may participate in the granzyme B apoptotic pathways. It is expressed in most tissues. CASP10 is also a less preferred gene symbol for what is now designated PPIG, see there. 

Molecular pathology
Defects in CASP10 have been linked to gastric cancer, non-Hodgkin lymphoma and autoimmune lymphoproliferative syndrome type 2A.
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References in periodicals archive
HLH-related genes (including AP3B1, FASLG, PRF1, TNFRSF1A, BIRC4, ITK, RAB27A, UNC13D, CASP10, ITPKC, SH2D1A, CD27, LYST, STX11, FAS, MAGT1 and STXBP2) were detected with high-throughput sequencing.
Rueff, "Genetic susceptibility in acute pancreatitis: genotyping of GSTM1, GSTT1, GSTP1, CASP7, CASP8, CASP9, CASP10, LTA, TNFRSF1B, and TP53 gene variants," Pancreas, vol.
In this experimental setting, we used, in addition to a pan-caspase inhibitor (Z-VAD-FMK), inhibitors of CASP8 (Z-IETD-FMK), CASP9 (Z-LEHD-FMK), and caspase-10 (CASP10; Z-AEVD-FMK) (R&D Systems, Minneapolis, MN, USA).
Inhibition of the extrinsic pathway by CASP10 and CASP8 inhibitors prevented apoptosis by up to 75% and 60%, respectively, after treatment with viscum and by 50% and 30%, respectively, in viscumTT-treated cells.
Initiator caspases (CASP2, CASP8, CASP9, and CASP10) cleave inactive pro-forms of effector caspases, thereby activating them.
For example, somatic mutations in CASP3 were found in two of 129 NHL cases [130] and somatic mutations in CASP10 in 15% of 117 cases [131].
Rare ALPS patients carrymutations of FASLG coding for FAS-ligand, or CASP10 coding for caspase 10 involved in FAS signaling; a mutation of FASLG is carried also by [MRL.sup.gld/gld] mice showing a disease similar to that displayed by [MRL.sup.lpr/lpr] mice.
Can the results be applied to the local population (CASP10)?
Kang et al., "Inactivating mutations of CASP10 gene in non-Hodgkin lymphomas," Blood, vol.
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