HSPD1

60 kDa heat shock protein, mitochondrial ;
60 kDa chaperonin ;
Chaperonin 60 ;
CPN60 ;
Heat shock protein 60 ;
HSP-60 ;
Hsp60 ;
HuCHA60 ;
Mitochondrial matrix protein P1 ;
P60 lymphocyte protein ;

disease:Defects in HSPD1 are a cause of spastic paraplegia autosomal dominant type 13 (SPG13) [MIM:605280]. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs.,disease:Defects in HSPD1 are the cause of leukodystrophy hypomyelinating type 4 (HLD4) [MIM:612233]; also called mitochondrial HSP60 chaperonopathy or MitCHAP-60 disease. HLD4 is 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 occurrs within the first 2 decades of life.,function:Implicated in mitochondrial protein import and macromolecular assembly. May facilitate 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.,similarity:Belongs to the chaperonin (HSP60) family.,similarity:Belongs to the TCP-1 chaperonin family.,subunit:Interacts with HBV protein X and HTLV-1 protein p40tax.,

cell activation, cytokine production, regulation of cytokine production, positive regulation of cytokine production,somatic diversification of immune receptors, somatic recombination of immunoglobulin genes during immune response,somatic diversification of immunoglobulins during immune response, activation of innate immune response, pattern recognition receptor signaling pathway, toll-like receptor signaling pathway, adaptive immune response, immune effector process, activation of immune response, cytokine production during immune response, B cell cytokine production, immunoglobulin production, immunoglobulin production during immune response, production of molecular mediator of immune response, leukocyte mediated immunity, lymphocyte mediated immunity, adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains,immune system development, somatic diversification of immune receptors via germline recombination within a single locus, positive regulation of immune system process, regulation of leukocyte activation, positive regulation of leukocyte activation, regulation of immune effector process, positive regulation of immune effector process, regulation of leukocyte mediated immunity, positive regulation of leukocyte mediated immunity, regulation of lymphocyte mediated immunity, positive regulation of lymphocyte mediated immunity, regulation of T cell mediated immunity,positive regulation of T cell mediated immunity, MyD88-dependent toll-like receptor signaling pathway, immune response-activating signal transduction, innate immune response-activating signal transduction, immune response-regulating signal transduction, regulation of adaptive immune response, positive regulation of adaptive immune response, regulation of adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains, positive regulation of adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains, regulation of response to biotic stimulus, positive regulation of response to biotic stimulus, regulation of response to tumor cell, positive regulation of response to tumor cell, regulation of immune response to tumor cell, positive regulation of immune response to tumor cell, regulation of T cell mediated immune response to tumor cell, positive regulation of T cell mediated immune response to tumor cell, DNA metabolic process, DNA recombination, protein folding, 'de novo' protein folding, protein complex assembly, apoptosis, activation of caspase activity, immune response, response to unfolded protein, cell death, cell proliferation, response to organic substance, posttranscriptional regulation of gene expression, regulation of cell death, positive regulation of cell death, positive regulation of peptidase activity, programmed cell death,immunoglobulin mediated immune response, death, somatic cell DNA recombination, somatic diversification of immunoglobulins, somatic recombination of immunoglobulin gene segments, B cell mediated immunity, positive regulation of defense response, regulation of protein stability, regulation of type I interferon production, positive regulation of type I interferon production, regulation of interferon-alpha production, regulation of interferon-gamma production, regulation of interleukin-10 production, regulation of interleukin-12 production, regulation of interleukin-6 production, positive regulation of interferon-alpha production, positive regulation of interferon-gamma production,positive regulation of interleukin-10 production, positive regulation of interleukin-12 production, positive regulation of interleukin-6 production, mononuclear cell proliferation, cellular macromolecular complex subunit organization, cellular macromolecular complex assembly, protein refolding, B cell proliferation, T cell activation, B cell activation, regulation of apoptosis, regulation of macrophage activation, positive regulation of macrophage activation, positive regulation of apoptosis, negative regulation of apoptosis, regulation of programmed cell death, positive regulation of programmed cell death, negative regulation of programmed cell death, positive regulation of catalytic activity, positive regulation of caspase activity, regulation of caspase activity, cellular protein complex assembly, macromolecular complex subunit organization, positive regulation of molecular function, regulation of innate immune response, positive regulation of innate immune response, isotype switching, leukocyte activation, lymphocyte activation, lymphocyte proliferation,isotype switching to IgG isotypes, positive regulation of response to stimulus, positive regulation of immune response,protein stabilization, regulation of T cell activation, regulation of cell activation, positive regulation of cell activation,positive regulation of T cell activation, chaperone-mediated protein complex assembly, positive regulation of multicellular organismal process, regulation of lymphocyte activation, positive regulation of lymphocyte activation,regulation of hydrolase activity, positive regulation of hydrolase activity, protein maturation, response to protein stimulus, regulation of peptidase activity, regulation of endopeptidase activity, negative regulation of cell death,macromolecular complex assembly, protein complex biogenesis, leukocyte proliferation,

Mitochondrion matrix.