antibody

VAPB (Vesicle-associated Membrane Protein-associated Protein B/C, VAMP-B/VAMP-C, VAMP-associated Protein B/C, VAP-B/VAP-C, UNQ484/PRO983)

MBS6011828

0.1 mg

655 USD

monoclonal

mouse

nonconjugated

[12K193]

Antibody

VAPB (Vesicle-associated Membrane Protein-associated Protein B/C, VAMP-B/VAMP-C, VAMP-associated Protein B/C, VAP-B/VAP-C, UNQ484/PRO983)

[VAPB]

[Anti -VAPB (Vesicle-associated Membrane Protein-associated Protein B/C, VAMP-B/VAMP-C, VAMP-associated Protein B/C, VAP-B/VAP-C, UNQ484/PRO983)]

[VAPB; Vesicle-associated membrane protein-associated protein B/C; vesicle-associated membrane protein-associated protein B/C; VAMP-associated 33 kDa protein; VAMP (vesicle-associated membrane protein)-associated protein B and C]

[VAPB; VAPB; ALS8; VAP-B; VAMP-B; VAMP-B/VAMP-C; VAMP-associated protein B/C; VAP-B/VAP-C]

VAPB_HUMAN

Monoclonal

IgG2B

[12K193]

Mouse

Human

Recognizes human VAP-B. Species sequence homology: recombinant rat VAP-B (50%). Does not react with recombinant human VAP-A.

Purified by Protein A affinity chromatography.

Supplied as a lyophilized powder in PBS, pH 6.4, 5% trehalose. Reconstitute with 200 ul sterile PBS.

Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile PBS. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Reconstituted product is stable for 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Immunohistochemistry (IHC)

Suitable for use in Immunohistochemistry. Dilution: Immunohistochemistry: 8-25ug/ml using immersion fixed paraffin-embedded sections of human brain (cerebellum). Optimal dilutions to be determined by the researcher.

Immunohistochemistry (IHC)

Immunogen: Recombinant corresponding to aa2-132 from human VAP-B expressed in E.coli.

Antibodies; Abs to Membrane Proteins

Vesicle-associated membrane protein associated protein B is an~30kD ubiquitously expressed type IV transmembrane protein belonging to the VAP family. It is found in endoplasmic reticulum (ER), Golgi and other membranes as a homodimer or a heterodimer with VAP-A, probably associating through a GxxxG motif in the transmembrane regions. Human VAP-B cDNA encodes 243aa that include a 222aa cytoplasmic domain and a 21aa C-terminal membrane anchor. The cytoplasmic domain contains a mobile sperm protein (MSP) domain aa7-124 and a coiled-coil region aa159-196. Human VAP-B shares 90%, 89%, 96%, 96% and 94% aa identity with mouse, rat, canine, bovine and porcine VAP-B, respectively. VAP-A and VAP-B MSP domains recruit FFAT motif-containing proteins to the cytosolic surface of ER membranes. FFAT proteins mediate many of the effects of VAPs on regulation of membrane transport, phospholipid biosynthesis, microtubule organization, and the unfolded protein response. VAPs also interact with some SNARE and viral proteins. A human polymorphism of VAP-B, P56S, is found in three familial motor neuron diseases, notably the amylotrophic lateral sclerosis variant ALS8. It produces a non-functional protein that can dimerize with and inhibit function of normal VAP-B, leading formation of intracellular aggregates and increased ER-stress-induced death of motor neurons. It can also promote cleavage and secretion of soluble VAP-B, which can then function as a ligand for EPH receptors. A naturally occurring 99aa isoform of VAP-B that diverges at 71aa within the MSP domain is termed VAP-C. It also appears to be a negative regulator of VAP-A and VAP-B. While VAP-B is used by hepatitis C virus (HCV) for its propagation, VAP-C inhibits HCV propagation.

37182052

AAQ88829.1

O95292

27,228 Da

Metabolism Pathway (477135); Metabolism Of Lipids And Lipoproteins Pathway (160976); Sphingolipid De Novo Biosynthesis Pathway (530750); Sphingolipid Metabolism Pathway (119543)

The protein encoded by this gene is a type IV membrane protein found in plasma and intracellular vesicle membranes. The encoded protein is found as a homodimer and as a heterodimer with VAPA. This protein also can interact with VAMP1 and VAMP2 and may be involved in vesicle trafficking. [provided by RefSeq, Jul 2008]

VAPB: Participates in the endoplasmic reticulum unfolded protein response (UPR) by inducing ERN1/IRE1 activity. Involved in cellular calcium homeostasis regulation. Defects in VAPB are the cause of amyotrophic lateral sclerosis type 8 (ALS8). ALS8 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. 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 cases leading to familial forms. Defects in VAPB are a cause of spinal muscular atrophy proximal adult autosomal dominant (SMAPAD); also called late-onset spinal muscular atrophy Finkel type. A form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. SMAPAD is characterized by proximal muscle weakness that begins in the lower limbs and then progresses to upper limbs, onset in late adulthood (after third decade) and a benign course. Most of the patients remain ambulatory 10 to 40 years after clinical onset. Belongs to the VAMP-associated protein (VAP) (TC 9.B.17) family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Membrane protein, integral. Chromosomal Location of Human Ortholog: 20q13.33. Cellular Component: Golgi membrane; Golgi apparatus; endoplasmic reticulum membrane; endoplasmic reticulum; integral to membrane. Molecular Function: protein binding; enzyme binding; protein homodimerization activity; FFAT motif binding; protein heterodimerization activity; microtubule binding; beta-tubulin binding. Biological Process: endoplasmic reticulum organization and biogenesis; cellular calcium ion homeostasis; ER to Golgi vesicle-mediated transport; positive regulation of viral genome replication; sphingolipid metabolic process; unfolded protein response, activation of signaling protein activity; sphingolipid biosynthetic process; unfolded protein response; virus-host interaction; negative regulation of viral protein levels in host cell. Disease: Spinal Muscular Atrophy, Late-onset, Finkel Type; Amyotrophic Lateral Sclerosis 8

0.1 mg

655 USD