TSC1 antibody (AA 401-430)

Catalog No. ABIN652207

Product Details anti-TSC1 Antibody

Target: TSC1 (Tuberous Sclerosis 1 (TSC1))

Binding Specificity: AA 401-430

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This TSC1 antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Purification: This antibody is purified through a protein A column, followed by peptide affinity purification.

Immunogen: This Hamartin (TSC1) antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 401-430 amino acids from the Central region of human Hamartin (TSC1).

Clone: RB11648

Isotype: Ig Fraction

Application Details

Application Notes: IF: 1:10~50. WB: 1:1000. IHC-P: 1:10~50

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C,-20 °C

Storage Comment: Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Expiry Date: 6 months

Target Details for TSC1

Target: TSC1 (Tuberous Sclerosis 1 (TSC1))

Alternative Name: Hamartin (TSC1) (TSC1 Products)

Synonyms: LAM antibody, TSC antibody, hamartin antibody, mKIAA0243 antibody, tsc1 antibody, wu:fc38b04 antibody, fa99f04 antibody, wu:fa99f04 antibody, zgc:63657 antibody, TSC complex subunit 1 antibody, tuberous sclerosis 1 antibody, TSC complex subunit 1b antibody, TSC complex subunit 1a antibody, TSC1 antibody, Tsc1 antibody, tsc1b antibody, tsc1 antibody, tsc1a antibody

Background: 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 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.

Molecular Weight: 129767

Gene ID: 7248

NCBI Accession: NP_000359, NP_001155898, NP_001155899

UniProt: Q92574

Pathways: RTK Signaling, AMPK Signaling, Regulation of Cell Size, Tube Formation