Hamartin (TSC1) Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IF, WB
Recommend Dilution	Western blot (1 µg/ml, detects a band of approximately 130 kDa in mouse EL4 cell lysates). Immunocytochemistry. Has been reported as suitable for use in IHC on mouse EL4 cell cytospins at 2 µg/ml.
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Immunogen	A 15 amino acid peptide located near the centre of human TSC1
Specificity	This antibody recognises an epitope within the internal region of the 130 kDa tumour suppressor protein, Tumour sclerosis complex 1 (TSC1).
Formulation	Phosphate buffered saline, 0.02 % Sodium Azide State: Aff - Purified State: Liquid Ig fraction
Concentration	lot specific
Purification	Affinity chromatography
Conjugation	Unconjugated
Storage Condition	Store the antibody at 2 - 8 °C up to one month or (in aliquots) at -20 °C for longer. Avoid repeated freezing and thawing. Should this product contain a precipitate we recommend microcentrifugation before use.
Gene Name	tuberous sclerosis 1
Database Link	Entrez Gene 7248 Human UniProt ID Q92574
Background	Mutation within the TSC1 gene is responsible for Tuberous sclerosis (TSC), an autosomal dominant genetic disorder associated with the development of benign tumors (hamartomas), a condition also induced by mutation of the Tumour sclerosis complex 2 (TSC2) gene. Many proteins have been shown to play a role in the regulation of the serine/threonine kinase known as target of rapamycin (TOR), a central component in a complex signaling pathway which controls cell proliferation and cell cycle progression. Negative regulation of TOR activity occurs following the over-expression of the TSC1 and TSC2 gene products, hamartin and tuberin. These act by suppressing the phosphorylation of eukaryotic translation initiation factor 4E binding protein 1 (4EBP1) and ribosomal protein S6 kinase (S6K), downstream targets of TOR involved in mRNA translation. Furthermore the TOR activator protein Rheb has been identified as a target of TSC1-TSC2 complexes.
Synonyms	KIAA0243, TSC, Tuberous sclerosis 1 protein
Reference Data

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