TSC1 Mouse anti-Human, Clone: OTI2B2, lyophilized, TrueMAB™

Catalog No.	Quantity
50-166-5817	100 μg

Catalog No. 50-166-5817 Supplier OriGene Supplier No. CF802012

Description

Mouse Monoclonal Antibody

Reconstitute with PBS (pH 7.3) and recommend to perform another round of desalting process using Product No. 7KMWCO

Implicated as a tumor suppressor. May have a function in vesicular transport. 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 domit multi-system disorder that affects especially the brain, kidneys, heart, and skin. TSC is characterized by hamartomas (benign overgrowths predomitly 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.

Specifications

• Antigen: TSC1
• Applications: Western Blot
• Classification: Monoclonal
• Clone: OTI2B2
• Conjugate: Unconjugated
• Formulation: PBS with 8% trehalose and no preservative; pH 7.3
• Gene: TSC1
• Gene Accession No.: Q92574
• Gene Alias: LAM, TSC
• Gene Symbols: TSC1
• Host Species: Mouse
• Immunogen: Human recombit protein fragment corresponding to amino acids 836-1164 of human TSC1 produced in E.coli.
• Purification Method: Affinity Chromatography
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 7248
• Target Species: Human
• Content And Storage: -20° C, Avoid Freeze/Thaw Cycles
• Product Type: Antibody
• Form: Lyophilized
• Isotype: IgG2a