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TARDBP Rabbit pAb (bs-0822R)  
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產(chǎn)品編號 bs-0822R
英文名稱 TARDBP Rabbit pAb
中文名稱 Tar DNA 結(jié)合蛋白43/TDP-43抗體
別    名 TAR DNA-binding protein 43; TAR DNA binding protein 43; ALS10; OTTHUMP00000002171; TAR DNA binding protein 43; TAR DNA binding protein; TDP 43; TDP-43; TDP43; tar DNA binding protein; ALS10; TADBP_HUMAN.  
研究領域 神經(jīng)生物學  信號轉(zhuǎn)導  細菌及病毒  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat (predicted: Human,Mouse,Rabbit,Cow,Chicken)
產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 45 kDa
檢測分子量
細胞定位 細胞核 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human TDP-43: 21-120/414 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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]

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 of cystic fibrosis. May also be involved in microRNA biogenesis, apoptosis and cell division. Can repress HIV-1 transcription by binding to the HIV-1 long terminal repeat. Stabilizes the low molecular weight neurofilament (NFL) mRNA through a direct interaction with the 3' UTR.

Subcellular Location:
Nucleus. In patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis, it is absent from the nucleus of affected neurons but it is the primary component of cytoplasmic ubiquitin-positive inclusion bodies.

Tissue Specificity:
Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.

Post-translational modifications:
Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Ubiquitinated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.
Cleaved to generate C-terminal fragments in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU.

DISEASE:
Defects in TARDBP are the 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. The disease is inherited in 5-10% of the cases.

Similarity:
Contains 2 RRM (RNA recognition motif) domains.

SWISS:
Q13148

Gene ID:
23435

Database links:

Entrez Gene: 23435 Human

Entrez Gene: 230908 Mouse

Entrez Gene: 298648 Rat

Omim: 605078 Human

SwissProt: Q13148 Human

SwissProt: Q921F2 Mouse

Unigene: 300624 Human

Unigene: 635053 Human

Unigene: 22453 Mouse

Unigene: 2633 Rat



變異蛋白質(zhì)TDP-43 在額顳葉退行性病變(FTLD-U)和萎縮性側(cè)索硬化癥(ALS)中表達較高。TDP-43在大腦中堆積能導致神經(jīng)細胞衰竭,從而引發(fā)疾病肌萎縮性側(cè)索硬化(ALS,也被稱為Lou Gehrig氏?。?br /> TDP-43這種癡呆是由大腦額葉的退化引起的,退化能延伸到顳葉。這是僅次于阿爾默海茲癥的讓65歲以下患者癡呆的第二種最常見的原因,通常影響40幾歲和50幾歲的人。TDP-43過去在神經(jīng)退化疾病患者病理的錯誤折疊蛋白中缺失。識別出這個蛋白應該有助于癡呆以及運動神經(jīng)元疾病的研究。
產(chǎn)品圖片
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-TARDBP Polyclonal Antibody, Unconjugated(bs-0822R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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