Rabbit Anti-VAPB/FITC Conjugated antibody

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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 a

Cat:

SL4227R-FITC

Species Reactivity：

Rat,(predicted: Human,Mouse,Chicken,Pig,Cow,Horse,Rabbit,Sheep,)

Immunogen：

KLH conjugated synthetic peptide derived from human VAPB

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

27kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
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:18596]; 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:18596]: 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.

Database links:

Entrez Gene: 9217 Human

Entrez Gene: 56491 Mouse

Entrez Gene: 60431 Rat

Omim: 605704 Human

SwissProt: O95292 Human

SwissProt: Q9QY76 Mouse

SwissProt: Q9Z269 Rat

Unigene: 182625 Human

Unigene: 260456 Mouse

Unigene: 52500 Rat

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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