TSC1 anticorps (AA 401-430)

N° du produit ABIN652207

Cet anticorps Lapin Polyclonal détecte spécifiquement TSC1 dans WB, IF et IHC (p). Il présente une réactivité envers Humain.

Aperçu rapide pour TSC1 anticorps (AA 401-430) (ABIN652207)

Antigène: TSC1 (Tuberous Sclerosis 1 (TSC1))

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp TSC1 est non-conjugé

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

Clone: RB11648

Détail du produit anti-TSC1 anticorps

Épitope: AA 401-430

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

Immunogène: 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).

Isotype: Ig Fraction

Information d'application

Indications d'application: IF: 1:10~50. WB: 1:1000. IHC-P: 1:10~50

Restrictions: For Research Use only

Stockage

Format: Liquid

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

Agent conservateur: Sodium azide

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

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Date de péremption: 6 months

Détails sur TSC1

Antigène: TSC1 (Tuberous Sclerosis 1 (TSC1))

Autre désignation: Hamartin (TSC1)

Sujet: 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.

Poids moléculaire: 129767

ID gène: 7248

NCBI Accession: NP_000359, NP_001155898, NP_001155899

UniProt: Q92574

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