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Anti-VCP Antibody

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产品名称: Anti-VCP Antibody
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Anti-VCP Antibody


Anti-VCP Antibody  的详细介绍
Name: Anti-VCP Antibody
See all VCP primary antibodies
Description: Rabbit polyclonal antibody to VCP.
Applications: WB, IHC, IF, IP
Dilutions: WB: 1:500 - 1:2000, IHC: 1:50 - 1:100, IF: 1:50 - 1:100, IP: 1:20 - 1:50.
Reactivity: Human, Mouse, Rat
Immunogen: A synthetic peptide of human VCP.
Protein Length: 806
Host: Rabbit
Clonality: Polyclonal
Isotype: IgG
Conjugate: Unconjugated
Purification: Affinity purification.
Product Form: Liquid
Formulation: Supplied in Phosphate Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 50% Glycerol.
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Function: Necessary for the fragmentation of Golgi stacks during mitosis and for their reassembly after mitosis. Involved in the formation of the transitional endoplasmic reticulum (tER). The transfer of membranes from the endoplasmic reticulum to the Golgi apparatus occurs via 50-70 nm transition vesicles which derive from part-rough, part-smooth transitional elements of the endoplasmic reticulum (tER). Vesicle budding from the tER is an ATP-dependent process. The ternary complex containing UFD1L, VCP and NPLOC4 binds ubiquitinated proteins and is necessary for the export of misfolded proteins from the ER to the cytoplasm, where they are degraded by the proteasome. The NPLOC4-UFD1L-VCP complex regulates spindle disassembly at the end of mitosis and is necessary for the formation of a closed nuclear envelope. Regulates E3 ubiquitin-protein ligase activity of RNF19A. Component of the VCP/p97-AMFR/gp78 complex that participates in the final step of the sterol-mediated ubiquitination and endoplasmic reticulum-associated degradation (ERAD) of HMGCR. Also involved in DNA damage response: recruited to double-strand breaks (DSBs) sites in a RNF8- and RNF168-dependent manner and promotes the recruitment of TP53BP1 at DNA damage sites (PubMed:22020440, PubMed:22120668). Recruited to stalled replication forks by SPRTN: may act by mediating extraction of DNA polymerase eta (POLH) to prevent excessive translesion DNA synthesis and limit the incidence of mutations induced by DNA damage (PubMed:23042607, PubMed:23042605). Required for cytoplasmic retrotranslocation of stressed/damaged mitochondrial outer-membrane proteins and their subsequent proteasomal degradation (PubMed:16186510, PubMed:21118995). Essential for the maturation of ubiquitin-containing autophagosomes and the clearance of ubiquitinated protein by autophagy (PubMed:20104022). Acts as a negative regulator of type I interferon production by interacting with DDX58/RIG-I: interaction takes place when DDX58/RIG-I is ubiquitinated via 'Lys-63'-linked ubiquitin on its CARD domains, leading to recruit RNF125 and promote ubiquitination and degradation of DDX58/RIG-I (PubMed:26471729).
Involvement in Disease: Inclusion body myopathy with early-onset Paget disease with or without frontotemporal dementia 1: An autosomal dominant disease characterized by disabling muscle weakness clinically resembling to limb girdle muscular dystrophy, osteolytic bone lesions consistent with Paget disease, and premature frontotemporal dementia. Clinical features show incomplete penetrance.

Amyotrophic lateral sclerosis 14, with or without frontotemporal dementia: A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. Patients with ALS14 may develop frontotemporal dementia.

Charcot-Marie-Tooth disease 2Y: An autosomal dominant, axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Neuropathies of the CMT2 group are characterized by signs of axonal degeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy.
Sequence Similarities: Belongs to the AAA ATPase family.
Post-Translational Modification: Phosphorylated by tyrosine kinases in response to T-cell antigen receptor activation. Phosphorylated in mitotic cells.
Cellular Location: Cytoplasm > Cytosol. Endoplasmic reticulum. Nucleus.

Present in the neuronal hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients. Present in the Lewy bodies specifically found in neurons from Parkinson disease patients. Recruited to the cytoplasmic surface of the endoplasmic reticulum via interaction with AMFR/gp78. Following DNA double-strand breaks, recruited to the sites of damage. Recruited to stalled replication forks via interaction with SPRTN.
Database Links:
  • Entrez Gene: 7415?Human
  • Entrez Gene: 269523?Mouse
  • Entrez Gene: 116643?Rat
  • Omim: 601023?Human
  • SwissProt: P55072?Human
  • SwissProt: Q01853?Mouse
  • SwissProt: P46462?Rat
  • Unigene: 529782?Human
  • Unigene: 245976?Mouse
  • Unigene: 98891?Rat
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    Synonyms:
  • 15S Mg(2+) ATPase p97 subunit Antibody
  • 15S Mg(2+)-ATPase p97 subunit Antibody
  • ALS14 Antibody
  • ATPase p97 Antibody
  • CDC48 Antibody
  • IBMPFD Antibody
  • MGC131997 Antibody
  • MGC148092 Antibody
  • MGC8560 Antibody
  • p97 Antibody
  • TER ATPase Antibody
  • TERA Antibody
  • TERA_HUMAN Antibody
  • Transitional endoplasmic reticulum ATPase Antibody
  • Valosin containing protein Antibody
  • Valosin-containing protein Antibody
  • VCP Antibody
  • Yeast Cdc48p homolog Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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