Rabbit anti-Human Hamartin Polyclonal Antibody | anti-TSC1 antibody

Hamartin, Phosphorylated (Ser505) (TSC1, Tuberous Sclerosis 1 Protein, KIAA0243, TSC) (APC)

Cat. Num. AAA6479420

• Gene Names: TSC1; LAM; TSC
• Reactivity: Human
• Applications: Dot Blot
• Purity: Purified by Immunoaffinity chromatography.
• Synonyms: Hamartin; Polyclonal Antibody; Phosphorylated (Ser505) (TSC1; Tuberous Sclerosis 1 Protein; KIAA0243; TSC) (APC); anti-TSC1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human TSC1 when phosphorylated at Ser505.
• Purity/Purification: Purified by Immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).
• Sequence Length: 1164

• Applicable Applications for anti-TSC1 antibody: FLISA, Dot Blot (DB)
• Application Notes: FLISA: 1:1000; Dot Blot: 1:500; Applications are based on unconjugated antibody.
• Immunogen: Synthetic phosphopeptide corresponding to amino acid residues surrounding Ser505 of human TSC1 (KLH).
• Conjugate: APC
• Antigen Modification: Phosphorylated
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Do not freeze APC conjugates. Light sensitive. Aliquots are stable for at least 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Dot Blot (DB)

(Dot blot analysis of anti-TSC1-pS505 Phospho-specific Pab (RB13337) on nitrocellulose membrane. 50ng of Phospho-peptide or Non Phospho-peptide per dot were adsorbed. Antibody working concentrations are 0.5ug per ml.)

Product Categories/Family for anti-TSC1 antibody

Antibodies; Disease Markers

References

1. Wu, J., et al., J. Cutan. Pathol. 31(5):383-387 (2004). 2. Lewis, J.C., et al., J. Med. Genet. 41(3):203-207 (2004). 3. J, et al., J. Child Neurol. 19(2):102-106 (2004). 4. Murthy, V., et al., J. Biol. Chem. 279(2):1351-1358 (2004). 5. Astrinidis, A., et al., J. Biol. Chem. 278(51):51372-51379 (2003).

NCBI and Uniprot Product Information

• NCBI GI #: 4507693
• NCBI GeneID: 7248
• NCBI Accession #: NP_000359
• NCBI GenBank Nucleotide #: NP_000359.1
• UniProt Accession #: Q92574
• Molecular Weight: 129.767
• NCBI Official Full Name: hamartin isoform 1
• NCBI Official Synonym Full Names: TSC complex subunit 1
• NCBI Official Symbol: TSC1
• NCBI Official Synonym Symbols: LAM; TSC
• NCBI Protein Information: hamartin
• UniProt Protein Name: Hamartin
• Protein Family: Hamartin
• UniProt Gene Name: TSC1
• UniProt Synonym Gene Names: KIAA0243; TSC
• UniProt Entry Name: TSC1_HUMAN

NCBI Description

This gene is a tumor suppressor gene that encodes the growth inhibitory protein hamartin. The encoded protein interacts with and stabilizes the GTPase activating protein tuberin. This hamartin-tuberin complex negatively regulates mammalian target of rapamycin complex 1 (mTORC1) signalling which is a major regulator of anabolic cell growth. This protein also functions as a co-chaperone for Hsp90 that inhibits its ATPase activity. This protein functions as a facilitator of Hsp90-mediated folding of kinase and non-kinase clients, including Tsc2 and thereby preventing their ubiquitination and proteasomal degradation. Mutations in this gene have been associated with tuberous sclerosis. [provided by RefSeq, Apr 2018]

Uniprot Description

TSC1: tuberous sclerosis protein 1 (TSC1). May have tumor suppressor activity. Together with tuberin (TSC2), inhibits mammalian target of rapamycin (mTOR)-mediated signaling to eukaryotic initiation factor 4E-binding protein 1 (4E-BP1) and ribosomal protein S6 kinase. Regulated by the phosphatidylinositol 3-kinase/Akt pathway and phosphorylation of tuberin. Phosphorylated by cyclin-dependent kinase 1/cyclin B during the cell cycle.

Protein type: Tumor suppressor; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 9q34

Cellular Component: TSC1-TSC2 complex; growth cone; protein complex; membrane; intracellular membrane-bound organelle; lamellipodium; cytoplasm; plasma membrane; actin filament; cell cortex; cytosol

Molecular Function: protein binding; chaperone binding; protein N-terminus binding; GTPase activating protein binding; GTPase regulator activity

Biological Process: myelination; regulation of cell cycle; protein heterooligomerization; regulation of cell-matrix adhesion; cell-matrix adhesion; negative regulation of insulin receptor signaling pathway; negative regulation of cell size; regulation of phosphoprotein phosphatase activity; cardiac muscle cell differentiation; response to insulin stimulus; regulation of translation; negative regulation of cell proliferation; positive regulation of focal adhesion formation; cell projection organization and biogenesis; synapse organization and biogenesis; cell cycle arrest; kidney development; potassium ion transport; negative regulation of TOR signaling pathway; protein stabilization; hippocampus development; rRNA export from nucleus; negative regulation of translation; glucose import; regulation of stress fiber formation; neural tube closure; insulin receptor signaling pathway; cerebral cortex development; regulation of protein kinase activity

Disease: Lymphangioleiomyomatosis; Focal Cortical Dysplasia Of Taylor; Tuberous Sclerosis 1