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ZMPSTE24

Domain

The metalloprotease domain is constituted by the two C-terminal nuclear regions.

Function

Transmembrane metalloprotease whose catalytic activity is critical for processing lamin A/LMNA on the inner nuclear membrane and clearing clogged translocons on the endoplasmic reticulum (PubMed:33293369, PubMed:33315887). Proteolytically removes the C-terminal three residues of farnesylated proteins (PubMed:33293369, PubMed:33315887). Plays also an antiviral role independently of its protease activity by restricting enveloped RNA and DNA viruses, including influenza A, Zika, Ebola, Sindbis, vesicular stomatitis, cowpox, and vaccinia (PubMed:28169297, PubMed:28246125). Mechanistically, controls IFITM antiviral pathway to hinder viruses from breaching the endosomal barrier by modulating membrane fluidity (PubMed:35283811).

Involvement in disease

Mandibuloacral dysplasia with type B lipodystrophy

MADB

A form of mandibuloacral dysplasia, a rare progeroid disorder with clinical and genetic heterogeneity, characterized by growth retardation, craniofacial dysmorphic features due to distal bone resorption, musculoskeletal and skin abnormalities associated with lipodystrophy. MADB is a disease characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and generalized lipodystrophy with loss of subcutaneous fat from the extremities, face, neck and trunk.

None

The disease is caused by variants affecting the gene represented in this entry.

Restrictive dermopathy 1

RSDM1

An autosomal recessive form of restrictive dermopathy, a genodermatosis mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial dysmorphism, sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life.

None

The disease is caused by variants affecting the gene represented in this entry.

Sequence similarities

Belongs to the peptidase M48A family.

Tissue specificity

Widely expressed. High levels in kidney, prostate, testis and ovary.

Cellular localization

  • Endoplasmic reticulum membrane
  • Multi-pass membrane protein
  • Nucleus inner membrane
  • Multi-pass membrane protein
  • Early endosome membrane
  • Multi-pass membrane protein
  • Late endosome membrane
  • Multi-pass membrane protein

Alternative names

  • FACE1
  • STE24
  • FACE1
  • STE24
  • ZMPSTE24
  • CAAX prenyl protease 1 homolog
  • Farnesylated proteins-converting enzyme 1
  • Prenyl protein-specific endoprotease 1
  • Zinc metalloproteinase Ste24 homolog
  • FACE-1

Target type

Proteins

Molecular weight

54813Da

We found 1 product in 1 category

Primary Antibodies

Target

Application

Reactive species

Search our catalogue for 'ZMPSTE24' (1)

Products