nucleoprotein TPR (TPR)

Function

• component of the cytoplasmic fibrils of the nuclear pore complex implicated in nuclear protein import
• its N-terminus is involved in activation of oncogenic kinases
• role in the mitotic spindle checkpoint
• phosphorylated upon DNA damage, probably by ATM or ATR
• interacts with MAD1L1 & MAD2L1

Compartment

• nucleus, nuclear pore complex
• nuclear membrane
• peripheral membrane protein, cytoplasmic side
• detected at kinetochores during prometaphase

Expression

• highest in testis, lung, thymus, spleen & brain
• lower levels in heart, liver & kidney

Pathology

• chromosomal rearrangements involving TPR are a cause of thyroid papillary carcinoma; intrachromosomal rearrangement that links the 5'-end of the TPR gene to the protein kinase domain of NTRK1 forms the fusion protein TRK-T1; TRK-T1 is a 55 kD protein reacting with antibodies against the carboxy terminus of the NTRK1 protein
• involved in tumorigenic rearrangements with the MET or RAF genes

More general terms

• proto oncogene protein
• nuclear protein
• phosphoprotein

Database

• UniProt: http://www.uniprot.org/uniprot/P12270.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7175
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7175
• OMIM: https://mirror.omim.org/entry/188550
• OMIM: https://mirror.omim.org/entry/189940