Abcam, ab234832, Anti-ZMPSTE24 antibody

Size: 100µL
Rabbit Polyclonal ZMPSTE24 antibody. Suitable for IHC-P, ICC/IF and reacts with Human samples. Cited in 1 publication. Immunogen corresponding to Recombinant Fragment Protein within Human ZMPSTE24 aa 200-350.
Key facts
Host species:Rabbit,
Clonality:Polyclonal,
Isotype:IgG,
Carrier free:No,
Reacts with:Human,
Applications:IHC-P, ICC/IFSee reactivity dataSee the reactivity data table below for information on validated species and application combinations.,
Immunogen:Recombinant Fragment Protein within Human ZMPSTE24 aa 200-350. The exact immunogen used to generate this antibody is proprietary information.O75844

Properties and Storage Information:
Form-Liquid, Purification technique-Affinity purification Protein G, Purification notes-Purity <95%., Storage buffer-pH: 7.4Preservative: 0.03% Proclin 300Constituents: PBS, 50% Glycerol (glycerin, glycerine), Shipped at conditions-Blue Ice, Appropriate short-term storage duration-1-2 weeks, Appropriate short-term storage conditions-+4°C, Appropriate long-term storage conditions--20°C, Aliquoting information-Upon delivery aliquot, Storage information-Avoid freeze / thaw cycle

Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
ZMPSTE24 also known as FACE1 or STE24 is a zinc metalloprotease enzyme involved in the post-translational modification of prelamin A to mature lamin A. This enzyme specifically cleaves the C-terminal tail of prelamin A a critical step required for proper nuclear envelope structure and function. ZMPSTE24 has a molecular mass of approximately 54 kDa and is expressed in several tissues including skeletal muscle heart and skin. Its activity primarily takes place in the endoplasmic reticulum membrane where it executes its proteolytic functions.
Biological function summary
The alteration of prelamin A to lamin A by ZMPSTE24 is necessary for maintaining nuclear shape and stability. Lamin A is a major component of the nuclear lamina providing structural support to the nuclear envelope. ZMPSTE24 is part of a proteolytic complex that ensures the proper maturation of lamin A which plays a significant role in DNA replication and cellular division. Deficiency in ZMPSTE24 activity can lead to the accumulation of farnesylated prelamin A disrupting nuclear architecture and cellular processes.
Pathways
The function of ZMPSTE24 integrates into the broader context of the lamin A maturation pathway significant for nuclear integrity. It collaborates with other enzymes like farnesyltransferase and icmt during the maturation process of prelamin A to lamin A. ZMPSTE24 also interacts with the protein lamin B which is an associate in the nuclear lamina. This cooperation within the pathway ensures the functional execution of nuclear mechanical properties and DNA repair processes.
The abnormal function or mutation of ZMPSTE24 connects direly with disorders such as restrictive dermopathy and Hutchinson-Gilford progeria syndrome (HGPS). In HGPS defective ZMPSTE24 fails to process prelamin A leading to the build-up of a toxic version of prelamin A termed progerin. This accumulation contributes to premature aging phenotypes. The disorder correlates with mutations in LMNA a gene encoding lamins A and C highlighting the tandem dependency between ZMPSTE24 and lamin A in maintaining nuclear structure and overall cellular health.