Hsp60 ist ein Gen, das durch das Symbol HSPD1 kodiert wird. Im Allgemeinen auch bezeichnet als: 60 kDa heat shock protein, mitochondrial; 60 kDa chaperonin; Chaperonin 60; CPN60; Heat shock protein 60; HSP-60; HuCHA60; Mitochondrial matrix protein P1; P60 lymphocyte protein; HSPD1. Hsp60 hat eine Masse von 61.06kDa, eine Aminosäurelänge von 573, und ist an folgenden Krankheiten beteiligt: Spastic paraplegia 13, autosomal dominant; Leukodystrophy, hypomyelinating, 4.
Wir bieten 60 antikörper gegen Hsp60, aufgewachsen in Kaninchen, Maus, Ziege und Huhn, welche geeignet sind für WB, IHC, ELISA, ICC/IF, FC and IP mit Proben abgeleitet von Human, Maus, Ratte, Rind, Schwein, Kaninchen, Affe, Schaf, Huhn, Hund, Drosophila, E. coli, Meerschweinchen, Hamster, Pferd und Xenopus.
Gen- und Proteininformationen
UniProt Zusammenfassung
Chaperonin implicated in mitochondrial protein import and macromolecular assembly. Together with Hsp10, facilitates the correct folding of imported proteins. May also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix (PubMed:1346131, PubMed:11422376). The functional units of these chaperonins consist of heptameric rings of the large subunit Hsp60, which function as a back-to-back double ring. In a cyclic reaction, Hsp60 ring complexes bind one unfolded substrate protein per ring, followed by the binding of ATP and association with 2 heptameric rings of the co-chaperonin Hsp10. This leads to sequestration of the substrate protein in the inner cavity of Hsp60 where, for a certain period of time, it can fold undisturbed by other cell components. Synchronous hydrolysis of ATP in all Hsp60 subunits results in the dissociation of the chaperonin rings and the release of ADP and the folded substrate protein (Probable).
Entrez Zusammenfassung
This gene encodes a member of the chaperonin family. The encoded mitochondrial protein may function as a signaling molecule in the innate immune system. This protein is essential for the folding and assembly of newly imported proteins in the mitochondria. This gene is adjacent to a related family member and the region between the 2 genes functions as a bidirectional promoter. Several pseudogenes have been associated with this gene. Two transcript variants encoding the same protein have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 13.
Rolle bei Krankheiten
Spastic paraplegia 13, autosomal dominant: A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.
Leukodystrophy, hypomyelinating, 4: A severe autosomal recessive hypomyelinating leukodystrophy. Clinically characterized by infantile-onset rotary nystagmus, progressive spastic paraplegia, neurologic regression, motor impairment, profound mental retardation. Death usually occurs within the first two decades of life.
Sequenzähnlichkeiten
Belongs to the chaperonin (HSP60) family.
Zellort
Mitochondrion matrix.
