Rabbit Polyclonal Hamartin antibody. C-terminal. Suitable for WB, IHC-P and reacts with Human, Mouse samples. Immunogen corresponding to Recombinant Fragment Protein within Human Hamartin.
IgG
Rabbit
pH: 7
Preservative: 0.025% Proclin 300
Constituents: 79% PBS, 20% Glycerol (glycerin, glycerine)
Liquid
Polyclonal
WB | IHC-P | |
---|---|---|
Human | Tested | Expected |
Mouse | Expected | Tested |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info 1/500.00000 - 1/3000.00000 | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Mouse | Dilution info Use at an assay dependent concentration. | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Mouse | Dilution info 1/100.00000 - 1/1000.00000 | Notes - |
Species | Dilution info | Notes |
---|---|---|
Species Human | Dilution info Use at an assay dependent concentration. | Notes - |
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Non-catalytic component of the TSC-TBC complex, a multiprotein complex that acts as a negative regulator of the canonical mTORC1 complex, an evolutionarily conserved central nutrient sensor that stimulates anabolic reactions and macromolecule biosynthesis to promote cellular biomass generation and growth (PubMed:12172553, PubMed:12271141, PubMed:12906785, PubMed:15340059, PubMed:24529379, PubMed:28215400). The TSC-TBC complex acts as a GTPase-activating protein (GAP) for the small GTPase RHEB, a direct activator of the protein kinase activity of mTORC1 (PubMed:12906785, PubMed:15340059, PubMed:24529379). In absence of nutrients, the TSC-TBC complex inhibits mTORC1, thereby preventing phosphorylation of ribosomal protein S6 kinase (RPS6KB1 and RPS6KB2) and EIF4EBP1 (4E-BP1) by the mTORC1 signaling (PubMed:12271141, PubMed:24529379, PubMed:28215400, PubMed:33215753). The TSC-TBC complex is inactivated in response to nutrients, relieving inhibition of mTORC1 (PubMed:12172553, PubMed:24529379). Within the TSC-TBC complex, TSC1 stabilizes TSC2 and prevents TSC2 self-aggregation (PubMed:10585443, PubMed:28215400). Acts as a tumor suppressor (PubMed:9242607). Involved in microtubule-mediated protein transport via its ability to regulate mTORC1 signaling (By similarity). Also acts as a co-chaperone for HSP90AA1 facilitating HSP90AA1 chaperoning of protein clients such as kinases, TSC2 and glucocorticoid receptor NR3C1 (PubMed:29127155). Increases ATP binding to HSP90AA1 and inhibits HSP90AA1 ATPase activity (PubMed:29127155). Competes with the activating co-chaperone AHSA1 for binding to HSP90AA1, thereby providing a reciprocal regulatory mechanism for chaperoning of client proteins (PubMed:29127155). Recruits TSC2 to HSP90AA1 and stabilizes TSC2 by preventing the interaction between TSC2 and ubiquitin ligase HERC1 (PubMed:16464865, PubMed:29127155).
KIAA0243, TSC, TSC, KIAA0243, TSC1, Hamartin, Tuberous sclerosis 1 protein
Rabbit Polyclonal Hamartin antibody. C-terminal. Suitable for WB, IHC-P and reacts with Human, Mouse samples. Immunogen corresponding to Recombinant Fragment Protein within Human Hamartin.
IgG
Rabbit
pH: 7
Preservative: 0.025% Proclin 300
Constituents: 79% PBS, 20% Glycerol (glycerin, glycerine)
Liquid
Polyclonal
Affinity purification Immunogen
Blue Ice
1-2 weeks
+4°C
-20°C
Upon delivery aliquot
Avoid freeze / thaw cycle
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This supplementary information is collated from multiple sources and compiled automatically.
The protein 'Hamartin' also known as TSC1 plays a mechanical role in cellular processes primarily as a regulator of cellular growth and proliferation. It interacts with Tuberin (TSC2) to form a complex that inhibits the small GTPase Rheb thereby controlling the mTOR signaling pathway. Hamartin has a molecular mass of approximately 130 kDa. It is expressed in various tissues including the brain heart and kidneys highlighting its significance in multiple physiological processes.
Hamartin functions as part of the TSC1-TSC2 complex which acts as a tumor suppressor. This complex inhibits the mechanistic target of rapamycin complex 1 (mTORC1) an essential regulator of cell growth proliferation and protein synthesis. Through this regulation Hamartin plays a critical role in maintaining cellular homeostasis and preventing abnormal cell growth that can lead to tumorigenesis.
Hamartin is integral to the mTOR signaling pathway an important pathway that regulates cell metabolism growth and survival. This pathway involves the interaction of Hamartin with Tuberin (TSC2) helping to control the activation of mTORC1. Additionally Hamartin is involved in the PI3K/Akt signaling pathway which further influences mTOR activity and cellular responses to growth factors.
Disruptions in Hamartin function are linked to Tuberous Sclerosis Complex (TSC) a genetic disorder characterized by tumor development in multiple organs. Mutations in the TSC1 gene lead to the loss of Hamartin function resulting in uncontrolled cell growth. Additionally research indicates that Hamartin may have a connection to certain epilepsy types given its expression in the brain and its role in neuronal development. In these conditions the interaction of Hamartin with Tuberin is essential for regulating cellular behavior and preventing disease progression.
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Paraffin-embedded mouse duodenum tissue stained for Hamartin using ab227594 at 1/500 dilution in immunohistochemical analysis.
5% SDS-PAGE gel.
All lanes: Western blot - Anti-Hamartin antibody - C-terminal (ab227594) at 1/1000 dilution
Lane 1: HEK-293T (human epithelial cell line from embryonic kidney transformed with large T antigen) whole cell extract at 30 µg
Lane 2: A431 (human epidermoid carcinoma cell line) whole cell extract at 30 µg
Lane 3: HeLa (human epithelial cell line from cervix adenocarcinoma) whole cell extract at 30 µg
Lane 4: HepG2 (human liver hepatocellular carcinoma cell line) whole cell extract at 30 µg
Predicted band size: 130 kDa
5% SDS-PAGE gel.
All lanes: Western blot - Anti-Hamartin antibody - C-terminal (ab227594) at 1/1000 dilution
Lane 1: U87-MG (human glioblastoma-astrocytoma epithelial cell line) whole cell extract at 30 µg
Lane 2: SK-N-SH (human neuroblastoma cell line) whole cell extract at 30 µg
Lane 3: IMR32 (human brain neuroblast cell line) whole cell extract at 30 µg
Lane 4: SK-N-AS (human brain neuroblastoma cell line) whole cell extract at 30 µg
Predicted band size: 130 kDa
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