transglutaminase-1, keratinocyte transglutaminase, epidermal transglutaminase, or transglutaminase-K

Function

• Ca+2-dependent activity
• catalyzes the cross-linking of proteins & the conjugation of polyamines to proteins
• responsible for cross- linking epidermal proteins during formation of the stratum corneum
• membrane anchorage region possesses a cluster of 5 Cys within which fatty acid(s) may become thioester-linked
• subject to phorbol ester-stimulated phosphorylation & is hypersensitive to proteolysis, which releases the enzyme in a soluble form

protein glutamine + alkylamine
               <-->
protein N(5)-alkylglutamine + NH3

Cofactor: binds 1 Ca+2 per subunit

Structure

• belongs to the transglutaminase superfamily transglutaminase family

Compartment

membrane, lipid-anchor

Pathology

• defects in TGM1 are the cause of
  • ichthyosis lamellar type 1
  • nonbullous congenital ichthyosiform erythroderma

More general terms

• transglutaminase
• phosphoprotein

Additional terms

• nonbullous congenital ichthyosiform erythroderma 1

Database

• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7051
• OMIM: https://mirror.omim.org/entry/190195
• OMIM: https://mirror.omim.org/entry/242100
• OMIM: https://mirror.omim.org/entry/242300
• UniProt: http://www.uniprot.org/uniprot/P22735.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7051