Transthyretin L55P Variant Monomers
Product Sizes
100 ug
SPR-451-100UG
2 x 100 ug
SPR-451-2X100UG
5 x 100 ug
SPR-451-5X100UG
About this Product
- SKU:
- SPR-451
- Additional Names:
- Transthyretin L55P, TTR L55P, ATTR, Amyloid polyneuropathy, Amyloidosis I, Carpal tunnel syndrome 1, CTS, CTS1, HEL111, HsT2651, PALB, Prealbumin, Prealbumin amyloidosis type I, Prealbumin Thyroxine-binding, TBPA, Thyroxine binding prealbumin, TTHY_HUMAN, TTR
- Application:
- Cell-based/Functional Assay, SDS-PAGE, Western Blot
- Buffer:
- PBS
- CE/IVD:
- RUO
- Extra Details:
- The L55P variant of transthyretin (TTR), a tetrameric transport protein for thyroxine and retinol, is one of the most pathogenic mutations linked to hereditary amyloidosis and other neurodegenerative disorders. This single amino acid substitution destabilizes the native tetrameric structure, promoting dissociation into monomers that readily misfold and aggregate into amyloid fibrils under physiological conditions. Unlike wild-type TTR, the L55P variant forms amyloidogenic monomers that serve as precursors to protofilaments and mature fibrils. These aggregates are implicated in transthyretin amyloid polyneuropathy (ATTR-PN), where they deposit in peripheral nerves and disrupt cellular integrity. The monomeric intermediate exhibits altered tertiary structure and increased B Beta-sheet content, facilitating its transition into neurotoxic assemblies. Recent studies reveal that L55P monomers compromise the blood-nerve barrier and trigger neuroinflammatory responses, exacerbating neuronal damage. Their ability to form protofilaments at neutral pH underscores their pathological relevance in vivo, distinguishing them from other TTR variants that require acidic conditions for fibril formation. Understanding the structural dynamics and aggregation pathways of L55P monomers is critical for developing targeted therapies. Stabilizing the tetrameric form or inhibiting monomer misfolding offers promising strategies to mitigate amyloid deposition and neurodegeneration. In summary, TTR L55P variant monomers are potent initiators of amyloid pathology and neurotoxicity, making them key targets in the quest to understand and treat transthyretin-related neurodegenerative diseases.
- Immunogen:
- Transthyretin (TTR) L55P Monomers
- Molecular Weight:
- 13.876 kDa
- Purity:
- >95%
- Purification:
- Ion Exchange
- Sequence:
- MGPTGTGESKCPLMVKVLDAVRGSPAINVAVHVFRKAADDTWEPFASGKTSESGEPHGLTTEEEFVEGIYKVEIDTKSYWKALGISPFHEHAEVVFTANDSGPRRYTIAALLSPYSYSTTAVVTNPKE
- Shipping Conditions:
- Dry Ice
- Storage Conditions:
- -70[o]C
- Supplier:
- StressMarq Biosciences
- Type:
- Proteins, Peptides, Small Molecules & Other Biomolecules: Recombinant Proteins
- Manufacturer's Data Sheet:https://www.stressmarq.com/products/protein/ttr-protein-spr-451/
