Human VAPB Recombinant Protein (RPPB5111)

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SKU:
RPPB5111
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
VAPB
Synonyms:
Vesicle-associated membrane protein-associated protein B/C
Synonyms:
VAMP-B/VAMP-C
Synonyms:
VAMP-associated protein B/C
Synonyms:
VAP-B/VAP-C
Source:
Escherichia Coli
Uniprot:
O95292
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Description

Product Name:Human VAPB Recombinant Protein
Product Code:RPPB5111
Size:20µg
Species:Human
Target:VAPB
Synonyms:Vesicle-associated membrane protein-associated protein B/C, VAMP-B/VAMP-C, VAMP-associated protein B/C, VAP-B/VAP-C, VAPB, ALS8, VAP-B, VAMP-B.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The VAPB solution (1 mg/ml) contains 20mM Tris-HCl buffer (pH8.0) and 10% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MAKVEQVLSL EPQHELKFRG PFTDVVTTNL KLGNPTDRNV CFKVKTTAPR RYCVRPNSGI IDAGASINVS VMLQPFDYDP NEKSKHKFMV QSMFAPTDTS DMEAVWKEAK PEDLMDSKLR CVFELPAEND KPHDVEINKI ISTTASKTET PIVSKSLSSS LDDTEVKKVM EECKRLQGEV QRLREENKQF KEEDGLRMRK TVQSNSPISA LAPTGKEEGL ST

VAPB Vesicle-associated membrane protein (VAMP)-associated protein B (aka VAPB) is a type IV transmembrane protein and belongs to the VAP family of proteins. VAPB may have a role in vesicle trafficking. VAPB is found in plasma and intracellular vesicle membranes as a homodimer and heterodimer with VAPA, and interacts with VAMP1 and VAMP2. VAPB defects are the basis for the amyotrophic lateral sclerosis type 8 and spinal muscular atrophy autosomal dominant Finkel type.

VAPB Human Recombinant fused with a 20 amino acid His tag at N-terminus produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 242 amino acids (1-222 a.a.) and having a molecular mass of 27.1kDa (molecular size on SDS-PAGE will appear higher).  The VAPB is purified by proprietary chromatographic techniques.

UniProt Protein Function: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.
UniProt Protein Details:

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; sphingolipid metabolic process; positive regulation of viral genome replication; 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

NCBI Summary: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]
UniProt Code:O95292
NCBI GenInfo Identifier:24638339
NCBI Gene ID:9217
NCBI Accession:O95292.3
UniProt Secondary Accession:O95292,O95293, Q9P0H0, A2A2F2,
UniProt Related Accession:O95292
Molecular Weight:243
NCBI Full Name:Vesicle-associated membrane protein-associated protein B/C
NCBI Synonym Full Names:VAMP (vesicle-associated membrane protein)-associated protein B and C
NCBI Official Symbol:VAPB  
NCBI Official Synonym Symbols:ALS8; VAP-B; VAMP-B  
NCBI Protein Information:vesicle-associated membrane protein-associated protein B/C; VAMP-associated 33 kDa protein
UniProt Protein Name:Vesicle-associated membrane protein-associated protein B/C
Protein Family:Virulence-associated protein
UniProt Gene Name:VAPB  
UniProt Entry Name:VAPB_HUMAN
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