TSC1 Antikörper (AA 401-430)

Produktnummer ABIN652207

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch TSC1 in WB, IF und IHC (p). Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für TSC1 Antikörper (AA 401-430) (ABIN652207)

Target: TSC1 (Tuberous Sclerosis 1 (TSC1))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser TSC1 Antikörper ist unkonjugiert

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

Klon: RB11648

Produktdetails anti-TSC1 Antikörper

Bindungsspezifität: AA 401-430

Aufreinigung: 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).

Isotyp: Ig Fraction

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

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

Konservierungsmittel: Sodium azide

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: 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.

Haltbarkeit: 6 months

Details zu TSC1

Target: TSC1 (Tuberous Sclerosis 1 (TSC1))

Andere Bezeichnung: Hamartin (TSC1)

Hintergrund: 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.

Molekulargewicht: 129767

Gen-ID: 7248

NCBI Accession: NP_000359, NP_001155898, NP_001155899

UniProt: Q92574

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