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AB152772

Recombinant Human Hamartin protein (GST tag N-Terminus)

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Recombinant Human Hamartin protein (GST tag N-Terminus) is a Human Fragment protein, in the 166 to 274 aa range, expressed in Wheat germ, suitable for SDS-PAGE, ELISA, WB.

View Alternative Names

KIAA0243, TSC, TSC1, Hamartin, Tuberous sclerosis 1 protein

1 Images
SDS-PAGE - Recombinant Human Hamartin protein (GST tag N-Terminus) (AB152772)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human Hamartin protein (GST tag N-Terminus) (AB152772)

12.5% SDS-PAGE analysis of ab152772 stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

WB, ELISA, SDS-PAGE

applications

Biologically active

No

Accession

Q92574

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

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Complementary Products

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Primary Antibodies

AB270967

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20ul selling size|KO Validated|RabMAb|Recombinant|Trial Size

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Proteins & Peptides

AB114179

Recombinant Human mTOR protein (GST tag N-Terminus)

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AB167933

Recombinant Human S6K1 protein

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Sequence info

[{"sequence":"LKKPGHVAEVYLVHLHASVYALFHRLYGMYPCNFVSFLRSHYSMKENLETFEEVVKPMMEHVRIHPELVTGSKDHELDPRRWKRLETHDVVIECAKISLDPTEASYEDG","proteinLength":"Fragment","predictedMolecularWeight":"37.73 kDa","actualMolecularWeight":null,"aminoAcidEnd":274,"aminoAcidStart":166,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"Q92574","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

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.
Biological function summary

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.

Pathways

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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Specifications

Form

Liquid

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General info

Function

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).

Post-translational modifications

Phosphorylation at Ser-505 does not affect interaction with TSC2.. 'Lys-63'-linked ubiquitinated at Lys-30 by PELI1; the ubiquitination promotes TSC1/TSC2 complex stability.

Subcellular localisation

Lysosome membrane

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Product protocols

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Target data

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).
See full target information TSC1
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