catalog number :
MBS140342
products full name :
Mouse Anti Human Transthyretin
products short name :
Transthyretin
products name syn :
TTR Antibody; Transthyretin, Mouse Anti Human; Transthyretin; ATTR; Prealbumin; TBPA; TTR; PALB; HsT2651
other names :
transthyretin; Transthyretin; transthyretin; ATTR; carpal tunnel syndrome 1; epididymis luminal protein 111; prealbumin, amyloidosis type I; thyroxine-binding prealbumin; transthyretin; ATTR; Prealbumin; TBPA
other gene names :
TTR; TTR; CTS; CTS1; PALB; TBPA; HEL111; HsT2651; PALB
uniprot entry name :
TTHY_HUMAN
isotype :
IgG1 heavy chain and kappa light chain
purity :
TTR antibody was purified from mouse ascitic fluids by protein-G affinity chromatography.
form :
1mg/ml containing PBS, pH-7.4, & 0.1% Sodium Azide. Sterile filtered colorless solution.
storage stability :
For periods up to 1 month store at 4 degree C, for longer periods of time, store at -20 degree C. Prevent freeze thaw cycles. 12 months at -20 degree C. 1 month at 4 degree C.
tested application :
ELISA (EIA), Western Blot (WB)
app notes :
TTR antibody has been tested by ELISA and Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results. Recommended dilution range for Western blot analysis is 1:500 ~ 3000.Recommended starting dilution is 1:500.
other info2 :
Immunogen: Anti-human TTR mAb, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with recombinant human TTR amino acids 21-147 purified from E Coli.
products categories :
MONOCLONAL/POLYCLONAL ANTIBODIES, MISCELLANEOUS ANTIBODIES; Monoclonal Antibodies
products description :
Introduction: Transthyretin (TTR) is a circulating and abundant tetramer, which functions as a carrier protein of thyroid hormone and retinol binding protein. It is primarily produced in the liver, but also in the brain and eye. Mutations in the TTR gene cause the protein to dissociate into monomers that after misfolding assemble into amyloid fibrils deposits in a variety of organs. TTR is also the only gene known to be associated with familial TTR amyloidosis. The diseases that originate from mutations in the TTR gene include amyloidotic polyneuropathy, euthyroid hyperthyroxinaemia, amyloidotic vitreous opacities, cardiomyopathy, oculoleptomeningeal amyloidosis, meningocerebrovascular amyloidosis, carpal tunnel syndrome, etc.
ncbi acc num :
NP_000362.1
ncbi gb acc num :
NM_000371.3
ncbi mol weight :
15,887 Da
ncbi pathways :
Amyloids Pathway (366238); Disease Pathway (530764); Diseases Associated With Visual Transduction Pathway (771581); Extracellular Matrix Organization Pathway (576262); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Non-integrin Membrane-ECM Interactions Pathway (833810); Retinoid Cycle Disease Events Pathway (771582); Retinoid Metabolism And Transport Pathway (187208); Signal Transduction Pathway (477114); The Canonical Retinoid Cycle In Rods (twilight Vision) Pathway (771585)
ncbi summary :
This gene encodes transthyretin, one of the three prealbumins including alpha-1-antitrypsin, transthyretin and orosomucoid. Transthyretin is a carrier protein; it transports thyroid hormones in the plasma and cerebrospinal fluid, and also transports retinol (vitamin A) in the plasma. The protein consists of a tetramer of identical subunits. More than 80 different mutations in this gene have been reported; most mutations are related to amyloid deposition, affecting predominantly peripheral nerve and/or the heart, and a small portion of the gene mutations is non-amyloidogenic. The diseases caused by mutations include amyloidotic polyneuropathy, euthyroid hyperthyroxinaemia, amyloidotic vitreous opacities, cardiomyopathy, oculoleptomeningeal amyloidosis, meningocerebrovascular amyloidosis, carpal tunnel syndrome, etc. [provided by RefSeq, Jan 2009]
uniprot summary :
TTR: Thyroid hormone-binding protein. Probably transports thyroxine from the bloodstream to the brain. Defects in TTR are the cause of amyloidosis transthyretin-related (AMYL-TTR). A hereditary generalized amyloidosis due to transthyretin amyloid deposition. Protein fibrils can form in different tissues leading to amyloid polyneuropathies, amyloidotic cardiomyopathy, carpal tunnel syndrome, systemic senile amyloidosis. The disease includes leptomeningeal amyloidosis that is characterized by primary involvement of the central nervous system. Neuropathologic examination shows amyloid in the walls of leptomeningeal vessels, in pia arachnoid, and subpial deposits. Some patients also develop vitreous amyloid deposition that leads to visual impairment (oculoleptomeningeal amyloidosis). Clinical features include seizures, stroke-like episodes, dementia, psychomotor deterioration, variable amyloid deposition in the vitreous humor. Defects in TTR are a cause of hyperthyroxinemia dystransthyretinemic euthyroidal (HTDE). It is a condition characterized by elevation of total and free thyroxine in healthy, euthyroid persons without detectable binding protein abnormalities. Defects in TTR are a cause of carpal tunnel syndrome type 1 (CTS1). It is a condition characterized by entrapment of the median nerve within the carpal tunnel. Symptoms include burning pain and paresthesias involving the ventral surface of the hand and fingers which may radiate proximally. Impairment of sensation in the distribution of the median nerve and thenar muscle atrophy may occur. This condition may be associated with repetitive occupational trauma, wrist injuries, amyloid neuropathies, rheumatoid arthritis. Belongs to the transthyretin family. Protein type: Secreted; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 18q12.1. Cellular Component: extracellular space; protein complex; cytoplasm; extracellular region. Molecular Function: identical protein binding; protein binding; protein heterodimerization activity; hormone activity. Biological Process: phototransduction, visible light; extracellular matrix organization and biogenesis; transport; retinol metabolic process; retinoid metabolic process. Disease: Hyperthyroxinemia, Dystransthyretinemic; Carpal Tunnel Syndrome; Amyloidosis, Hereditary, Transthyretin-related