UNIPROT #

P02766

PROTEIN NAME

Prealbumin PA

SYNONYMS

Amyloid polyneuropathy antibody; Amyloidosis I antibody; ATTR antibody; Carpal tunnel syndrome 1 antibody; CTS antibody; CTS1 antibody; Dysprealbuminemic euthyroidal hyperthyroxinemia antibody; Dystransthyretinemic hyperthyroxinemia antibody; Epididymis luminal protein 111 antibody; HEL111 antibody; HsT2651 antibody; PALB antibody; Prealbumin amyloidosis type I antibody; Prealbumin antibody; Prealbumin Thyroxine-binding antibody; Senile systemic amyloidosis antibody; TBPA antibody; Thyroxine binding prealbumin antibody; Transthyretin antibody; TTHY_HUMAN antibody; TTR antibody; TTR protein antibody

SEQUENCE SIMILARITIES

Belongs to the transthyretin family.

TISSUE SPECIFICITY

Detected in serum and cerebrospinal fluid (at protein level). Highly expressed in choroid plexus epithelial cells. Detected in retina pigment epithelium and liver.

POST-TRANSLATIONAL MODIFICATION

Not glycosylated under normal conditions. Following unfolding, caused for example by variant AMYL-TTR 'Gly-38', the cryptic Asn-118 site is exposed and glycosylated by STT3B-containing OST complex, leading to its degradation by the ER-associated degradation (ERAD) pathway.

SUBCELLULAR LOCATION

Secretory protein

FUNCTION

Transthyretin (TTR) originally called prealbumin is a serum and cerebrospinal fluid carrier of the thyroid hormone thyroxine (T4) and retinol. Prealbumin concentrations are disproportionately high in human ventricular cerebrospinal fluid (CSF). Over 80 different disease-causing mutations in prealbumin have been reported. TTR is known to be associated with the amyloid diseases senile systemic amyloidosis (SSA), familial amyloid polyneuropathy (FAP), and familial amyloid cardiomyopathy (FAC).