Recombinant Mouse ATP-dependent zinc metalloprotease YME1L1 (Yme1l1)
Description
Introduction
Recombinant Mouse ATP-dependent Zinc Metalloprotease YME1L1 (Yme1l1) is an ATP-dependent metalloprotease that belongs to the AAA family of ATPases and is encoded by the nuclear genome . Embedded in the inner mitochondrial membrane, its protease domain faces the intermembrane space . YME1L1 plays a crucial role in maintaining mitochondrial function and morphology . It was initially identified in yeast through a screen for gene products that elevate the rate of mitochondrial DNA migration to the nucleus .
Structure and Function
YME1L1 is a nuclear-encoded protein that is imported into the mitochondria as a premature form of approximately 80 kDa . Upon entry, it undergoes proteolytic processing by the mitochondrial processing peptidase (MPP), which cleaves off the mitochondrial targeting sequence (MTS) to produce a mature protein of roughly 63 kDa . This mature form then assembles into a homo-oligomeric complex within the inner mitochondrial membrane .
YME1L1 has several key functions :
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Regulation of mitochondrial morphology and function through cleavage of OPA1 (optic atrophy 1) at position S2, which promotes the maintenance of normal mitochondrial structure and mitochondrial protein metabolism .
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Maintaining normal cristae morphology and complex I respiration activity .
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Promoting antiapoptotic activity and protecting mitochondria from the accumulation of oxidatively damaged membrane proteins .
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Controlling the accumulation of nonassembled respiratory chain subunits (NDUFB6, OX4, and ND1) .
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Catalyzing the degradation of OMA1 in response to membrane depolarization .
YME1L1 in Disease
Mutations in the YME1L1 gene can cause mitochondriopathies, which often manifest as multisystemic disorders affecting high-energy-consuming organs . A homozygous mutation in YME1L1 has been identified as the cause of a novel mitochondriopathy with infantile-onset developmental delay, muscle weakness, ataxia, and optic nerve atrophy .
YME1L1 and Mitochondrial Dynamics
YME1L1 is essential for regulating mitochondrial fission, function, and apoptosis . Knock-down of YME1L1 disrupts the balance of mitochondrial fusion and fission, leading to increased mitochondrial fission . This disruption can result in reduced ATP content and increased mitophagy, ultimately impairing embryo development .
Product Specs
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Target Background
- Studies in a mouse model of neonatal hypoxic-ischemic brain injury indicate alterations in the expression of mitochondrial shaping proteins, such as OPA1 and Yme1L; both in vitro and in vivo, OPA1 is cleaved into shorter forms, and Yme1L expression is reduced. PMID: 26393574
- Constitutive OPA1 cleavage by YME1L and OMA1 at two distinct sites results in the accumulation of both long and short OPA1 forms, maintaining mitochondrial fusion. PMID: 24616225
