Von Hippel Lindau Binding protein 1
Encyclopedia
von Hippel–Lindau binding protein 1 (VBP1), also known as "prefoldin
Prefoldin
Prefoldin is a family of proteins used in protein folding complexes. It is classified as a heterohexameric molecular chaperone in both archaea and eukarya, including humans. A prefoldin molecule works as a transfer protein in conjunction with a molecule of chaperonin to form a chaperone complex...

 3", is a chaperone protein that binds to von Hippel–Lindau protein and transports it from perinuclear granules to the nucleus
Cell nucleus
In cell biology, the nucleus is a membrane-enclosed organelle found in eukaryotic cells. It contains most of the cell's genetic material, organized as multiple long linear DNA molecules in complex with a large variety of proteins, such as histones, to form chromosomes. The genes within these...

 or cytoplasm
Cytoplasm
The cytoplasm is a small gel-like substance residing between the cell membrane holding all the cell's internal sub-structures , except for the nucleus. All the contents of the cells of prokaryote organisms are contained within the cytoplasm...

 inside the cell
Cell (biology)
The cell is the basic structural and functional unit of all known living organisms. It is the smallest unit of life that is classified as a living thing, and is often called the building block of life. The Alberts text discusses how the "cellular building blocks" move to shape developing embryos....

. It is also involved in transporting nascent polypeptides to cytosolic chaperonin
Chaperonin
Chaperonins are proteins that fold and unfold other proteins. Newly made proteins usually must fold from a linear chain of amino acids into a three-dimensional form. Chaperonins belong to a large class of molecules that assist protein folding, called molecular chaperones...

s for post-translational folding
Protein folding
Protein folding is the process by which a protein structure assumes its functional shape or conformation. It is the physical process by which a polypeptide folds into its characteristic and functional three-dimensional structure from random coil....

.

VBP1 is a 197–amino acid
Amino acid
Amino acids are molecules containing an amine group, a carboxylic acid group and a side-chain that varies between different amino acids. The key elements of an amino acid are carbon, hydrogen, oxygen, and nitrogen...

 heterohexamer comprising two prefoldin-α and four prefoldin-β subunits, and is a member of the prefoldin-α subunit family
Protein family
A protein family is a group of evolutionarily-related proteins, and is often nearly synonymous with gene family. The term protein family should not be confused with family as it is used in taxonomy....

. It is ubiquitously expressed in tissues, and is located in the cell in the nucleus and cytoplasm. The VBP1 gene is located at Xq28. Homologues
Homology (biology)
Homology forms the basis of organization for comparative biology. In 1843, Richard Owen defined homology as "the same organ in different animals under every variety of form and function". Organs as different as a bat's wing, a seal's flipper, a cat's paw and a human hand have a common underlying...

 are known to exist between human VBP1 and proteins in mice, Drosophila
Drosophila melanogaster
Drosophila melanogaster is a species of Diptera, or the order of flies, in the family Drosophilidae. The species is known generally as the common fruit fly or vinegar fly. Starting from Charles W...

and C. elegans
Caenorhabditis elegans
Caenorhabditis elegans is a free-living, transparent nematode , about 1 mm in length, which lives in temperate soil environments. Research into the molecular and developmental biology of C. elegans was begun in 1974 by Sydney Brenner and it has since been used extensively as a model...

.

See also

  • von Hippel–Lindau tumor suppressor
  • von Hippel–Lindau disease
  • Eugen von Hippel
    Eugen von Hippel
    Eugen von Hippel was a German ophthalmologist who was born in Königsberg. He studied medicine in Heidelberg under ophthalmologist Theodor Leber and neurologist Wilhelm Heinrich Erb . In 1897 he attained the title of "professor extraordinary" at Heidelberg, and in 1909 became a professor at the...

  • Arvid Lindau
    Arvid Lindau
    Arvid Lindau was a Swedish pathologist born in Malmö. During his career he published more than forty papers on pathology, neurology, and bacteriology. At the Institute of Pathological Anatomy in Lund he wrote an important thesis titled Studien über Kleinhirncysten...

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