PMID: 3756141

 

    Legend: Gene, Sites

Title : Human C1 inhibitor: primary structure, cDNA cloning, and chromosomal localization

Abstract :
  1. The primary structure of human C1 inhibitor was determined by peptide and DNA sequencing
  2. The single-chain polypeptide moiety of the intact inhibitor is 478 residues (52,869 Da), accounting for only 51% of the apparent molecular mass of the circulating protein (104,000 Da)
  3. The positions of six glucosamine-based and five galactosamine-based oligosaccharides were determined
  4. Another nine threonine residues are probably also glycosylated
  5. Most of the carbohydrate prosthetic groups (probably 17) are located at the amino-terminal end ( residues 1-120 ) of the protein and are particularly concentrated in a region where the tetrapeptide sequence Glx-Pro-Thr-Thr , and variants thereof, is repeated 7 times
  6. No phosphate was detected in C1 inhibitor
  7. Two disulfide bridges connect cysteine-101 to cysteine-406 and cysteine-108 to cysteine-183
  8. Comparison of the amino acid and cDNA sequences indicates that secretion is mediated by a 22-residue signal peptide and that further proteolytic processing does not occur
  9. C1 inhibitor is a member of the large serine protease inhibitor (serpin) gene family
  10. The homology concerns residues 120 through the C-terminus
  11. The sequence was compared with those of nine other serpins, and conserved and nonconserved regions correlated with elements in the tertiary structure of alpha 1-antitrypsin
  12. The C1 inhibitor gene maps to chromosome 11, p11 .2-q13
  13. C1 inhibitor genes of patients from four hereditary angioneurotic edema kindreds do not have obvious deletions or rearrangements in the C1 inhibitor locus
  14. A HgiAI DNA polymorphism, identified following the observation of sequence variants , will be useful as a linkage marker in studies of mutant C1 inhibitor genes
Output (sent_index, trigger, protein, sugar, site):
  • 4. glycosylated, , -, -, threonine residues
  • 5. located, , -, the carbohydrate prosthetic groups, residues 1-120
Output(Part-Of) (sent_index, protein, site):
  • 5. protein, residues 1-120
*Output_Site_Fusion* (sent_index, protein, sugar, site):

 

 

Protein NCBI ID SENTENCE INDEX
structure of alpha 1-antitrypsin 5265 11
11, p11 8909 12