ELK Biotechnology PABP2 rabbit pAb

제품명

PABP2 rabbit pAb

제품 정보

세포 내 위치

Nucleus, Cytoplasm, Nucleus speckle.
Localized in cytoplasmic mRNP granules containing untranslated mRNAs. Shuttles between the nucleus and the cytoplasm but predominantly found in the nucleus (PubMed:10688363).
Its nuclear import may involve the nucleocytoplasmic transport receptor transportin and a RAN-GTP-sensitive import mechanism (By similarity).
Is exported to the cytoplasm by a carrier-mediated pathway that is independent of mRNA traffic.
Colocalizes with SKIP and poly(A) RNA in nuclear speckles (By similarity).
Intranuclear filamentous inclusions or ‘aggregates’ are detected in the myocytes of patients; these inclusions contain PABPN1, ubiquitin, subunits of the proteasome and poly(A) RNA.

배경(Background)

This gene encodes an abundant nuclear protein that binds with high affinity to nascent poly(A) tails.
The protein is required for progressive and efficient polymerization of poly(A) tails at the 3′ ends of eukaryotic transcripts and controls the size of the poly(A) tail to about 250 nt.
At steady-state, this protein is localized in the nucleus whereas a different poly(A) binding protein is localized in the cytoplasm.
This gene contains a GCG trinucleotide repeat at the 5′ end of the coding region, and expansion of this repeat from the normal 6 copies to 8–13 copies leads to autosomal dominant oculopharyngeal muscular dystrophy (OPMD).
Related pseudogenes have been identified on chromosomes 19 and X.
Read-through transcription also exists between this gene and the neighboring upstream BCL2-like 2 (BCL2L2) gene.
[provided by RefSeq, Dec 2010]