Target intelligence / Profile preview

Serine protease FAM111B (FAM111B)

Target
FAM111B
Molecular classification
Enzyme (serine protease, trypsin-like domain), DNA repair–associated protein, Cancer-associated nucleoprotein
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Overview

Serine protease FAM111B is an uncharacterized human enzyme predicted to function in DNA repair, cell cycle regulation, and apoptosis. It contains a C-terminal trypsin-like serine protease domain and shows functional homology with FAM111A, a DNA-protein crosslink (DPC) protease. FAM111B degrades p16 (CDKN2A) to promote cell cycle progression, interacts with DNA replication and repair proteins such as PCNA and RFC1, and regulates G2/M transition via cyclin B1 and CDC25C. Dysregulation or mutation of FAM111B can lead to cancer progression and hereditary multisystem fibrosis (POIKTMP), highlighting its critical role in genome stability, DNA repair, and apoptosis. Currently, it is considered a potential therapeutic target in oncology and rare disease, but dedicated drugs and specific inhibitors have not been developed.

Other names
FAM111BCancer-associated nucleoprotein (CANP)Family with sequence similarity 111 member BPOIKTMP (based on disease: hereditary fibrosing poikiloderma with tendon contracture, myopathy, and pulmonary fibrosis)Serine protease FAM111B
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Mechanism of action

Hypothetical: Serine protease inhibitors could inhibit proteolytic activity, potentially restoring proper cell cycle control or blocking cancer progression—no direct clinical evidence. Interference with DNA repair pathways if the protease domain is targeted.

03

Biological functions

DNA repair (esp. DNA-protein crosslink removal)Cell cycle regulation (including progression through G1/S and G2/M phases)Apoptosis induction upon failure of DNA repair
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Disease associations

Cancer (overexpression associated with progression and poor prognosis)Fibrosis (mutations lead to POIKTMP: poikiloderma, tendon contracture, myopathy, pulmonary fibrosis)Genome instability syndromes
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Safety considerations

Therapeutic targeting could potentially disrupt normal DNA repair, cell cycle progression, and apoptosis, leading to risks of genomic instability or unwanted cell deathLoss-of-function mutations cause multisystem syndromes, indicating essential cellular roles
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Interacting drugs

No current approved drugs directly target FAM111B

2 more in the full profile.

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Biomarkers

FAM111B expression levels may serve as a prognostic indicator in cancer (high expression correlates with poor prognosis)Mutational status of FAM111B for POIKTMP diagnosis

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