Peroxisomal membrane protein PMP34

Peroxisomal membrane protein PMP34 (also known as PMP34) is a protein that in humans is encoded by the PEX11B gene. It is a member of the peroxisome family of proteins, which are essential for the normal functioning of cells.

Function

PMP34 is a peroxisomal membrane protein that plays a crucial role in the division and proliferation of peroxisomes. Peroxisomes are small, membrane-bound organelles that are involved in a variety of metabolic processes, including the breakdown of fatty acids and the detoxification of harmful substances. PMP34 is thought to regulate the number and size of peroxisomes within the cell, thereby influencing the cell's metabolic capacity.

Structure

The PMP34 protein is composed of 314 amino acids and has a molecular weight of approximately 34 kDa. It is an integral membrane protein, meaning it is embedded within the lipid bilayer of the peroxisomal membrane. The protein has three predicted transmembrane domains, which are regions of the protein that span the membrane.

Clinical significance

Mutations in the PEX11B gene, which encodes PMP34, have been associated with a number of peroxisomal disorders. These include Zellweger syndrome, a severe congenital disorder characterized by the absence or reduction in the number of peroxisomes, and adrenoleukodystrophy, a progressive neurodegenerative disorder that affects the white matter of the brain.

Research

Research into PMP34 and other peroxisomal proteins is ongoing, with the aim of better understanding the role of peroxisomes in health and disease. This research could potentially lead to new treatments for peroxisomal disorders and other conditions in which peroxisome function is impaired.

See also

• Peroxisome
• Zellweger syndrome
• Adrenoleukodystrophy

References

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