Page last updated: 2024-10-24

regulation of protein targeting to mitochondrion

Definition

Target type: biologicalprocess

Any process that modulates the frequency, rate or extent of protein targeting to mitochondrion. [GO_REF:0000058, GOC:bf, GOC:PARL, GOC:TermGenie]

Protein targeting to mitochondria is a complex and essential process that ensures the delivery of newly synthesized proteins to their correct destination within this organelle. Mitochondria are essential for cellular respiration and play a critical role in energy production, apoptosis, and other cellular processes. The correct localization of proteins to mitochondria is therefore crucial for maintaining cellular function.

The process begins with the synthesis of mitochondrial proteins in the cytoplasm. These proteins often contain specific targeting sequences, known as mitochondrial targeting sequences (MTSs), which are short, positively charged, and hydrophobic regions. MTSs act as “zip codes” directing the proteins to the mitochondria.

Once synthesized, mitochondrial proteins are recognized by chaperone proteins in the cytoplasm. These chaperones prevent the proteins from folding prematurely and facilitate their interaction with import machinery on the mitochondrial surface.

The mitochondrial outer membrane (MOM) contains a protein complex called the translocase of the outer membrane (TOM) complex. The TOM complex acts as a gatekeeper, recognizing MTSs and facilitating the passage of proteins across the MOM.

After crossing the MOM, proteins are further transported to the mitochondrial inner membrane (MIM) by the translocase of the inner membrane (TIM) complex. The TIM complex acts as a channel for the passage of proteins across the MIM.

Inside the mitochondrial matrix, the proteins are further processed by mitochondrial chaperones and proteases. These proteins assist in protein folding, remove MTSs, and ensure the proper localization of the proteins within the mitochondrial matrix, inner membrane, or intermembrane space.

Protein targeting to mitochondria is a highly regulated process that involves multiple steps and a variety of protein factors. This process is crucial for maintaining mitochondrial function and cellular viability. Dysregulation of this process can lead to various mitochondrial disorders and diseases.'
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Proteins (1)

ProteinDefinitionTaxonomy
Sterol regulatory element-binding protein 1A sterol regulatory element-binding protein 1 that is encoded in the genome of human. [PRO:CNA, UniProtKB:P36956]Homo sapiens (human)

Compounds (1)

CompoundDefinitionClassesRoles
calcifediolD3 vitamins;
diol;
hydroxycalciol
bone density conservation agent;
human metabolite;
metabolite;
mouse metabolite;
nutraceutical