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H1779-02D Goat Anti-Hamartin (TSC1, Tuberous Sclerosis 1) (Isoform 1)

Specifications
References
Clone Type
Polyclonal
Host
Goat
Source
Human
Isotype
IgG
Grade
Affinity Purified
Applications
E WB
Crossreactivity
Hu
Accession #
NP_000359.1
Shipping Temp
Blue Ice
Storage Temp
-20°C
tumor suppressor, tuberous sclerosis 1 protein, TSC, MGC86987, LAM, KIAA0243, HGNC:12362, tuberous sclerosis 1, hamartin, TSC1

This gene encodes a growth inhibitory protein thought to play a role in the stabilization of tuberin. Mutations in this gene have been associated with tuberous sclerosis. Alternative splicing results in multiple transcript variants.

Applications
Suitable for use in ELISA and Western Blot. Other applications have not been tested.
Recommended Dilutions
Peptide ELISA Titer: 1:128,000 Western Blot: 0.1-0.3ug/ml detects ~150kD band observed in Human Brain lysates (calculated MW of 130kD according to NP_000359.1). The observed molecular weight corresponds to earlier findings in literature with different antibodies (Miloloza et al Hum Mol Genet. 2000 Jul 22;9(12):1721-7; PMID: 10915759). In transfected HEK293 transiently expressing Human TSC1 a band of approx. 120kD is observed. This band is not observed in the non-transfected HEK293. Optimal dilutions to be determined by the researcher.
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Synthetic peptide corresponding to C-QLHIMDYNETHHEH, from the C Terminus of human Hamartin (NP_000359.1).
Form
Supplied as a liquid in Tris saline, pH 7.2, 0.5% BSA, 0.02% sodium azide.
Purity
Purified by peptide affinity chromatography.
Specificity
Recognizes human Hamartin. This antibody is expected to recognise tuberous sclerosis isoform 1 (NP_000359.1) only. Species sequence homology: mouse, rat, canine.
References
1. Chan JA, Zhang H, Roberts PS, Jozwiak S, Wieslawa G, Lewin-Kowalik J, Kotulska K, Kwiatkowski DJ. |Pathogenesis of tuberous sclerosis subependymal giant cell astrocytomas: biallelic inactivation of TSC1 or TSC2 leads to mTOR activation.J Neuropathol Exp Neurol. 2004 Dec;63(12):1236-42.
USBio References
No references available
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