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Translocation of Proteins into the Mitochondria01:19

Translocation of Proteins into the Mitochondria

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Mitochondrial precursors are translocated to the internal subcompartments via independent mechanisms involving distinct protein machineries called translocases.
Sorting of outer membrane proteins:
Mitochondrial outer membrane proteins are of two types: the transmembrane, beta-barrel porins, and the membrane-anchored, alpha-helical proteins. Beta-barrel porin precursors are translocated by the TOM complex and inserted into the outer mitochondrial membrane by the SAM complex. In contrast,...
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Intracellular Signaling Affects Focal Adhesions01:17

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Integrins act both as extracellular input receivers and as intracellular processing activators. As their name suggests, integrins are entirely integrated into the membrane structure. Their hydrophobic membrane-spanning regions interact with the phospholipid bilayer's hydrophobic region. These membrane receptors provide extracellular attachment sites for effectors like hormones and growth factors. They activate intracellular response cascades when their effectors are bound and active.
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Mitochondrial Precursor Proteins01:39

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Mitochondrial precursors are partially unfolded or loosely folded polypeptide chains. Newly synthesized precursors are inhibited from spontaneously folding into their native conformation by the cytosolic chaperones, heat shock proteins 70 (Hsp70), and mitochondrial import stimulation factors (MSFs). Precursors bound to MSFs are guided to the TOM70-TOM37 receptors, while precursors bound to Hsp70  chaperones are targetted to TOM20-TOM22 receptor complexes.
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Regulation of Angiogenesis and Blood Supply01:24

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Rapidly dividing tumors, embryos, and wounded tissues require more oxygen than usual, lowering the oxygen concentration in the blood. At low oxygen or hypoxic conditions, an oxygen-sensitive transcription factor called the hypoxia-inducible factor 1 or HIF1 is activated. HIF1 is a dimeric protein of alpha (ɑ) and beta (β) subunits.  Under optimal oxygen conditions, HIF1β is present in the nucleus while HIF1ɑ remains in the cytosol. HIF1ɑ is hydroxylated by prolyl...
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Energy to Drive Translocation01:37

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Mitochondrial protein import is powered by two distinct energy sources: ATP hydrolysis and electrochemical potential across the inner membrane. Newly synthesized precursors are bound by cytosolic chaperones of the Hsp70 family, which guide them to the import receptors on the mitochondrial surface. Utilizing the energy of ATP hydrolysis, Hsp70 chaperones transfer these precursors to the TOM receptors on the mitochondrial outer membrane.
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After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...
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Related Experiment Video

Updated: Feb 17, 2026

Detection of Mitochondria Membrane Potential to Study CLIC4 Knockdown-induced HN4 Cell Apoptosis In Vitro
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Adhesion Regulating Molecule 1 Mediates HAP40 Overexpression-Induced Mitochondrial Defects.

Zih-Ning Huang1, Her Min Chung1, Su-Chiung Fang2,3

  • 1Department of Life Sciences, College of Bioscience and Biotechnology, National Cheng Kung University, Tainan 70101, Taiwan.

International Journal of Biological Sciences
|December 7, 2017
PubMed
Summary

Huntingtin-associated protein 40 (HAP40) causes mitochondrial dysfunction in Huntington's disease by reducing adhesion regulating molecule 1 (ADRM1), leading to cell death. Restoring ADRM1 improves mitochondrial function and cell viability.

Keywords:
ADRM1Drp1.HAP40Huntington's diseasemitochondrial dynamics

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Area of Science:

  • Neuroscience
  • Cell Biology
  • Mitochondrial Biology

Background:

  • Huntington's disease (HD) involves striatal neuron death linked to mitochondrial dysfunction.
  • Mechanisms of mitochondrial dysregulation in HD are not fully understood.
  • Increased Huntingtin-associated protein 40 (HAP40) is observed in HD, but its role is unclear.

Purpose of the Study:

  • To investigate the role of HAP40 in mitochondrial dysfunction in Huntington's disease.
  • To elucidate the molecular mechanisms linking HAP40 to mitochondrial impairment.

Main Methods:

  • Overexpression of HAP40 in immortalized mouse striatal neurons.
  • siRNA-mediated depletion of adhesion regulating molecule 1 (ADRM1).
  • Assessment of mitochondrial function, ROS levels, and cell viability.
  • Analysis of dynamin-related GTPase protein 1 (Drp1) phosphorylation and activity.
  • Treatment with Drp1 inhibitor Mdivi-1.

Main Results:

  • HAP40 overexpression induced mitochondrial dysfunction and reduced cell viability.
  • HAP40-induced dysfunction correlated with decreased ADRM1 protein levels.
  • ADRM1 depletion impaired mitochondrial function and increased fragmentation.
  • Reduced ADRM1 enhanced Drp1 activity via increased Drp1 phosphorylation at Ser616.
  • Restoring ADRM1 ameliorated HAP40-induced mitochondrial issues.
  • Mdivi-1 treatment mitigated mitochondrial dysfunction caused by HAP40 or ADRM1 depletion.

Conclusions:

  • HAP40 reduction of ADRM1 contributes to mitochondrial fragmentation and dysfunction in HD models.
  • Altered mitochondrial fission activity, mediated by the HAP40-ADRM1-Drp1 axis, is a key factor in HD pathogenesis.
  • Targeting the HAP40-ADRM1 pathway may offer therapeutic strategies for Huntington's disease.