ELK Biotechnology Dipeptidyl-peptidase 1 (heavy chain, Cleaved-Leu231) rabbit pAb

제품명

Dipeptidyl-peptidase 1 (heavy chain, Cleaved-Leu231) rabbit pAb

공급업체

ELK Biotechnology

제품 정보

Background

• Catalytic activity: Releases N-terminal dipeptides (Xaa-Yaa-|Zaa-), except when Xaa is Arg or Lys, or Yaa/Zaa is Pro.
• Cofactor: Binds one chloride ion per heavy chain.
• Disease associations:
  • Defects in CTSC cause Haim-Munk syndrome (HMS) [MIM:245010], juvenile periodontitis (JPD) [MIM:170650], and Papillon-Lefevre syndrome (PLS) [MIM:245000].
  • HMS: Autosomal recessive disorder with palmoplantar keratosis, onychogryphosis, and periodontitis.
  • JPD: Autosomal dominant, severe gingival infections leading to tooth loss.
  • PLS: Autosomal recessive, palmoplantar keratosis and early tooth loss.
• Enzyme regulation: Strongly inhibited by cysteine peptidase inhibitors (mersalyl acid, iodoacetic acid, cystatin). Not inhibited by PMSF, bestatin, or metal ion chelators.
• Function: Thiol protease with dipeptidylpeptidase activity. Acts as exopeptidase and endopeptidase. Activates serine proteases (elastase, cathepsin G, granzymes A/B), neuraminidase, and factor XIII.
• Induction: Up-regulated in lymphocytes by IL-2.
• PTM: Cleavage at position 58 or 61 in ~50% of complexes; parts held by disulfide bond. N-glycosylated.
• Similarity: Belongs to peptidase C1 family.
• Subunit: Tetramer of heterotrimers (exclusion domain, heavy, and light chains).
• Tissue specificity: Ubiquitous; highly expressed in lung, kidney, and placenta; intermediate in colon, small intestine, spleen, and pancreas.

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