Autoimmune lymphoproliferative syndrome type II

Gene Information

Names

HUGO name: CASP10

Alias(es):

  • ALPS2
  • FLICE2
  • MCH4
  • FADD-like ICE2
  • Fas-associated death domain protein
  • ICE-like apoptotic protease 4
  • Apoptotic protease MCH-4
  • Caspase 10, apoptosis-related cysteine protease
  • Interleukin-1B-converting enzyme 2
  • Caspase-10 precursor
  • ICE-like apoptotic protease 4
  • Apoptotic protease MCH-4
  • Fas-associated death domain protein interleukin-1B-converting enzyme

Localization

Reference sequences

Chromosomal location

Maps

Markers

Protein Information

Description

Protein function:

Involved in the activation cascade of caspases responsible for apoptosis execution. Recruited to both FAS- and TNFR-1 receptors in a FADD dependent manner. May participate in the granzyme B apoptotic pathways. Cleaves and activates caspase-3, -4, -6, -7, -8, and -9. Hydrolyzes the small- molecule substrates, Tyr-Val-Ala-Asp-|-Amc and Asp-Glu-Val-Asp-|-Amc.

Subunit:

Heterodimer of a 23/17 kDa (p23/17) depending on the splicing events and a 12 kDa (p12) subunit.

Post-translational modification:

Cleavage by granzyme B and autocatalytic activity generate the two active subunits.

Tissue specificity:

Detectable in most tissues. Lowest expression is seen in brain, kidney, prostate, testis and colon.

Similarity:

Belongs to peptidase family C14.

Structures (PDB)

Expression

Expression pattern for human

Tissue Expression (%) Clones
uterus 25.68 1:1305
invasive adenocarcinoma 16.52 1:2028
leukopheresis 8.63 2:7769
cell line 8.52 1:3934
bone marrow 5.75 1:5829
liver 4.23 1:7919
dorsal root ganglia 4.16 1:8052
placenta 4.06 1:8261
colon tumor, RER+ 4.01 1:8360
pooled human melanocyte, fetal heart, and pregnant uterus 3.09 3:32508
uterus 25.68 1:1305
invasive adenocarcinoma 16.52 1:2028
leukopheresis 8.63 2:7769
cell line 8.52 1:3934
bone marrow 5.75 1:5829
liver 4.23 1:7919
dorsal root ganglia 4.16 1:8052
placenta 4.06 1:8261
colon tumor, RER+ 4.01 1:8360
pooled human melanocyte, fetal heart, and pregnant uterus 3.09 3:32508

Animal models

Mouse