基因名称(Gene Name)
TARDBP TDP43
蛋白名称
TAR DNA-binding protein 43 (TDP-43)
免疫原
Synthesized peptide derived from human protein . at AA range: 41-90
特异性
TADBP Monoclonal Antibody detects endogenous levels of protein.
组成
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse antiserum by affinity-chromatography using epitope-specific immunogen.
其他名称
SFTPA2; COLEC5; PSAP; SFTP1; SFTPA; SFTPA2B; Pulmonary surfactant-associated protein A2; PSP-A; PSPA; SP-A; SP-A2; 35 kDa pulmonary surfactant-associated protein; Alveolar proteinosis protein; Collectin-5; SFTPA1; COLEC4; PSAP;SFTP1; SFTPA; SFTPA1B; Pulmonary surfactant-associated protein A1; PSP-A; PSPA; SP-A; SP-A1; 35 kDa pulmonary surfactant-associated protein; Alveolar proteinosis protein; Collectin-4
背景
TAR DNA binding protein(TARDBP) Homo sapiens HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. Activation of HIV-1 gene expression by the transactivator Tat is dependent on an RNA regulatory element (TAR) located downstream of the transcription initiation site. The protein encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. A similar pseudogene is present on chromosome 20. [provided by RefSeq, Jul 2008],
功能
disease:Defects in TARDBP are a cause of amyotrophic lateral sclerosis type 10 (ALS10) [MIM:612069]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of ALS is likely to be multifactorial, involving both genetic and environmental factors. TARDBP is the primary component of ubiquitin-positive inclusion bodies found in ALS and in frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLDU).,function:DNA and RNA-binding protein which regulates transcription and splicing. Involved in the regulation of CFTR splicing. It promotes CFTR exon 9 skipping by binding to the UG repeated motifs in the polymorphic region near the 3'-splice site of this exon. The resulting aberrant splicing is associated with pathological features typical o
