Evidence map›Paper›PMID 40877583›Full record

ReviewFunctional & integrative genomics2025

MBTPS1: a membrane-bound transcription factor protease implicated in the pathogenesis of several skin and skeletal disorders.

Haisheng Huang, Yumeng Wang, Fuying Chen, Anqi Zhao, Wei He, Ming Li

Abstract readReview
PubMed Publisher
In one paragraph

Review in Functional & integrative genomics, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors.

Haisheng Huang *Anhui University of Science and Technology First Affiliated Hospital, Anhui, 232001, China.
Yumeng Wang *Department of Dermatology, The Children's Hospital of Fudan University, Shanghai, 201102, China.
Fuying ChenDepartment of Dermatology, The Children's Hospital of Fudan University, Shanghai, 201102, China.
Anqi ZhaoDepartment of Dermatology, The Children's Hospital of Fudan University, Shanghai, 201102, China.
Wei HeDepartment of Dermatology, The Children's Hospital of Fudan University, Shanghai, 201102, China.
Ming LiAnhui University of Science and Technology First Affiliated Hospital, Anhui, 232001, China. mingli@fudan.edu.cn.

Funding

National Nature Science Foundation of China 82473509 and 82073422
6 · The paper itself

Abstract

The MBTPS1 gene, which is located on chromosome 16q24, encodes the membrane-bound transcription factor protease site-1 (MBTPS1), commonly referred to as site-1 protease (S1P). S1P can process a variety of substrates independently or in conjunction with membrane-bound transcription factor protease site-2 (MBTPS2, also known as S2P), including sterol regulatory element binding proteins (SREBPs), activating transcription factor 6 (ATF6) and cyclic-AMP responsive element‑binding protein 3 (CREB3). Variants in the MBTPS1 gene can lead to multiple clinically distinct disorders with different phenotypes, including spondyloepiphyseal dysplasia of Kondo-Fu type (SEDKF), Cataract, alopecia, oral mucosal disorder, and psoriasis-like (CAOP) syndrome, and Silver-Russell-like syndrome (SRS). This review presents the structural and functional characteristics of S1P, enumerates the relevant substrates and elucidates the spectrum of associated disorders resulting from pathogenic variants of MBTPS1, and discusses the correlations investigates the genotype-phenotype correlations underlying these distinct clinical manifestations.

Indexed as

Bone DiseasesProprotein ConvertasesSerine EndopeptidasesSkin DiseasesAnimalsHumansLipid Metabolismmembrane-bound transcription factor peptidase, site 1Proprotein ConvertasesSerine EndopeptidasesCAOPGenetic Skin DiseasesMBTPS1S1PSEDKFSite-1SRS

Identifiers

What Socratic holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.