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Product Name VAPB Chinese Name 囊泡相关膜蛋白相关的蛋白B Alias ALS 8; ALS8; D2Abb2e; UNQ484/PRO983; Vamp 33b; VAMP associated 33 kDa protein; VAMP associated protein B and C; VAMP associated protein B; VAMP associated protein B/C; VAMP associated protein C; VAMP B; VAMP B VAMP C; VAMP B/VAMP C; VAMP C; VAMP vesicle associated membrane protein associated protein B and C; Vamp33b; VAMPB; VAMPB/VAMPC; VAMPC; VAP 33b; VAP B; VAP B/VAP C; VAP C; VAP33b; VAPB/VAPC; VAPC antibody Vesicle associated membrane protein associated protein B and C; Vesicle associated membrane protein associated protein B/C; VAPB_HUMAN. Research Area Tumour Cell biology immunology Signal transduction Immunogen Species Rabbit Clonality Polyclonal React Species Rat, (predicted: Human, Mouse, Chicken, Pig, Cow, Horse, Rabbit, Sheep, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 27kDa Cellular localization cytoplasmic Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human VAPB: 51-150/243 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail VAPB contains 1 MSP domain and it may play a role in vesicle trafficking. Defects in VAPB are a cause of proximal adult autosomal dominant spinal muscular atrophy [MIM:182980]; also called late onset spinal muscular atrophy Finkel type. Spinal muscular atrophies are neurodegenerative disorders characterized by degeneration of lower motor neurons, leading to progressive paralysis muscular atrophy. This form is a late adult onset form of the disease (after age 20 years). The patients show a benign course, most of them remaining ambulatory 10 to 40 years after clinical onset.
Function:
Participates in the endoplasmic reticulum unfolded protein response (UPR) by inducing ERN1/IRE1 activity. Involved in cellular calcium homeostasis regulation.
Subunit:
Homodimer, and heterodimer with VAPA. Interacts with VAMP1 and VAMP2. Interacts with HCV NS5A and NS5B. Interacts (via MSP domain) with ZFYVE27. Interacts with RMDN3.
Subcellular Location:
Endoplasmic reticulum membrane; Single-pass type IV membrane protein (By similarity). Note=Present in mitochondria-associated membranes that are endoplasmic reticulum membrane regions closely apposed to the outer mitochondrial membrane.
Tissue Specificity:
Ubiquitous. Isoform 1 predominates.
DISEASE:
Amyotrophic lateral sclerosis 8 (ALS8) [MIM:608627]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
Spinal muscular atrophy, proximal, adult, autosomal dominant (SMAPAD) [MIM:182980]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the VAMP-associated protein (VAP) (TC 9.B.17) family.
Contains 1 MSP domain.
SWISS:
O95292
Gene ID:
9217
Database links:Entrez Gene: 9217 Human
Entrez Gene: 56491 Mouse
Omim: 605704 Human
SwissProt: O95292 Human
SwissProt: Q9QY76 Mouse
Unigene: 182625 Human
Unigene: 260456 Mouse
Unigene: 52500 Rat
Product Picture Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (VAPB) Polyclonal Antibody, Unconjugated (SL4227R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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