anti-TSC1 antibody (Tuberous Sclerosis 1) (AA 401-430)

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Antigen
Synonyms tsc1, wu:fc38b04, LAM, TSC, hamartin, mKIAA0243
Alternatives
Epitope
AA 401-430, Center
(53), (40), (40), (40), (40), (27), (21), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (20), (17), (15), (12), (12), (12), (12), (8), (7), (7), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (6), (4), (4), (4), (4), (3), (3), (3), (2), (1), (1), (1), (1), (1)
Reactivity
Human
(426), (358), (153), (5), (1), (1), (1), (1), (1), (1)
Host
Rabbit
(728), (37), (19), (1)
Clonality (Clone)
Polyclonal ()
Conjugate
This TSC1 antibody is un-conjugated
(67), (67), (67), (64), (64), (64), (4), (3), (3), (3), (3), (3), (3), (3), (3)
Application
Immunofluorescence (IF), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Western Blotting (WB)
(532), (490), (153), (62), (34), (34), (17), (14), (7), (5), (4), (2), (2)
Pubmed 2 references available
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Quantity 400 μL
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Immunogen This Hamartin (TSC1) antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 401-430 AA from the Central region of human Hamartin (TSC1).
Clone RB11648
Isotype Ig
Specificity This TSC1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the Center region of human TSC1.
Purification This antibody is purified through a protein A column, followed by peptide affinity purification.
Alternative Name Hamartin (TSC1) (TSC1 Antibody Abstract)
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.
Synonyms: Hamartin, Tuberous sclerosis 1 protein, TSC1, KIAA0243, TSC
Molecular Weight 129767 DA
Gene ID 7248
UniProt Q92574
Research Area Cell Structure
Pathways RTK Signaling, AMPK Signaling
Application Notes WB = 1:1000, IHC (p) = 1:10-50, IF = 1:10-50
Restrictions For Research Use only
Format Liquid
Concentration 0.5 mg/mL
Buffer 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
Supplier Images
Western Blotting (WB) image for anti-TSC1 antibody (Tuberous Sclerosis 1) (AA 401-430) (ABIN652207) TSC1 Antibody (Center) (ABIN652207) western blot analysis in MDA-MB231 cell line lysa...
Immunohistochemistry (IHC) image for anti-TSC1 antibody (Tuberous Sclerosis 1) (AA 401-430) (ABIN652207) Hamartin (TSC1) Antibody (Center) (ABIN652207)immunohistochemistry analysis in formal...
Background publications Lewis, Thomas, Murphy et al.: "Genotype and psychological phenotype in tuberous sclerosis." in: Journal of medical genetics, Vol. 41, Issue 3, pp. 203-7, 2004 (PubMed).

Astrinidis, Senapedis, Coleman et al.: "Cell cycle-regulated phosphorylation of hamartin, the product of the tuberous sclerosis complex 1 gene, by cyclin-dependent kinase 1/cyclin B." in: The Journal of biological chemistry, Vol. 278, Issue 51, pp. 51372-9, 2003 (PubMed).

Catalog No. ABIN652207
$324.50
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Plus shipping costs $45.00

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