Interaction between TSC1 and TSC2 may facilitate vesicular docking.
Defects in TSC1 are the cause of tuberous sclerosis complex (TSC).
The molecular basis of TSC is a functional impairement of the hamartin-tuberin complex.
TSC is an autosomal dominant multi-system disorder that affects especially the brain, kidneys, heart, and skin.
TSC is characterized by hamartomas (benign overgrowths predominantly of a cell or tissue type that occurs normally in the organ) and hamartias (developmental abnormalities of tissue combination).
Clinical symptoms can range from benign hypopigmented macules of the skin to profound mental retardation with intractable seizures to premature death from a variety of disease-associated causes.
Defects in TSC1 may be a cause of focal cortical dysplasia of Taylor balloon cell type (FCDBC).
FCDBC is a subtype of cortical displasias linked to chronic intractable epilepsy.
Cortical dysplasias display a broad spectrum of structural changes, which appear to result from changes in proliferation, migration, differentiation, and apoptosis of neuronal precursors and neurons during cortical development.
Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, FLISA
Recommended Dilution:FLISA1:1,000 Western Blot: 1:100-500Immunohistochemistry: 1:10-50Immunofluorescence: 1:10-50
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
Caution: FITC conjugates are sensitive to light.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).
Specificity：Human
Isotype：IgG
Calc Applications Abbrev：FLISA IF IHC WB
Calc Crossreactivity：Hu
Immunogen：TSC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the Center region of human TSC1.
