TARDBP Ready-To-Use IHC Kit (免疫组化试剂盒) - IHC-P | Bioss
货号：IHC0429
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概述
产品编号
IHC0429
英文名称
TARDBP Ready-To-Use IHC Kit
中文名称
Tar DNA 结合蛋白43即用型免疫组化试剂盒
英文别名
1190002A23Rik; ALS10; C85084; TARDBP; TDP-43; TDP43; wu:fb77f02; wu:fc52g10
描述
IHC0429 is a ready-to-use IHC kit for staining of TARDBP. The kit provides all reagents, from antigen retrieval to cover slip mounting, that require little to no diluting or handling prior to use. Simply apply the reagents to your sample slide according to the protocol and you're steps away from obtaining high-quality IHC data.
适用样品基质
FFPE tissue
应用范围
Immunohistochemistry
保存条件
Please store components at the temperatures indicated on the individual tube labels. The kit is stable for 6 months from the date of receipt.
背景资料
TDP43 was first identified as a novel cellular protein that binds to HIV-1 virus TAR DNA sequence motifs and acts a transcriptional repressor to the HIV-1 LTR. Later experiments revealed that TDP43 also regulates the splicing of exon 9 of the cystic fibrosis transmembrane conductance regular (CFTR), most likely through the association with the UG repeats at the 3"e; splice site. A hyperphosphorylated, ubiquitinated, and cleaved form of TDP43 known as pathologic TDP43 is the major disease protein in ubiquitin-positive, tau-, and alpha-synuclein-negative frontotemporal dementia (FLTD-U). TDP43 is not related to TRBP1, and RNA binding protein that binds HIV-1 TAR RNA sequences.
产品应用
产品应用: IHC-P
交叉反应
交叉反应: Human, Mouse, Rat
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靶标
基因名
TARDBP
蛋白名
TAR DNA-binding protein 43
亚基
Homodimer (PubMed:20043239, PubMed:24464995).
Homooligomer (via its N-terminal domain) (PubMed:28663553, PubMed:29438978).
Interacts with BRDT (By similarity).
Binds specifically to pyrimidine-rich motifs of TAR DNA and to single stranded TG repeated sequences. Binds to RNA, specifically to UG repeated sequences with a minimum of six contiguous repeats. Interacts with ATXN2; the interaction is RNA-dependent (PubMed:20740007).
Interacts with MATR3 (PubMed:24686783).
Interacts with UBQLN2 (PubMed:23541532).
Interacts with HNRNPA2B1 (PubMed:19429692).
Interacts with ZNF106 (By similarity).
Interacts with CNOT7/CAF1 (PubMed:30520513).
Interacts with CRY2 (PubMed:27123980).
Interacts with PPIA/CYPA; the interaction is dependent on RNA-binding activity of TARDBP and PPIase activity of PPIA/CYPA and acetylation of PPIA/CYPA at 'Lys-125' favors the interaction (PubMed:25678563).
亚细胞定位
#Cytoplasm
#Mitochondrion
#Nucleus
组织特异性
Ubiquitously expressed. In particular, expression is high in pancreas, placenta, lung, genital tract and spleen.
翻译后修饰
Hyperphosphorylated in hippocampus, neocortex, and spinal cord from individuals affected with ALS and FTLDU. Phosphorylated upon cellular stress.
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.
疾病
Amyotrophic lateral sclerosis 10 (ALS10)
Note
The disease is caused by variants affecting the gene represented in this entry. Neurodegeneration is caused by activation of the cGAS-STING pathway: defects in TARDBP trigger mitochondrial DNA release into the cytosol via the permeability transition pore (PubMed:33031745). Released mitochondrial DNA is then detected by CGAS, leading to activation of the cGAS-STING pathway, triggering type-I interferon production and autoinflammation (PubMed:33031745)1 Publication
Description
A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.
功能
RNA-binding protein that is involved in various steps of RNA biogenesis and processing (PubMed:23519609).
Preferentially binds, via its two RNA recognition motifs RRM1 and RRM2, to GU-repeats on RNA molecules predominantly localized within long introns and in the 3'UTR of mRNAs (PubMed:23519609, PubMed:24240615, PubMed:24464995).
In turn, regulates the splicing of many non-coding and protein-coding RNAs including proteins involved in neuronal survival, as well as mRNAs that encode proteins relevant for neurodegenerative diseases (PubMed:21358640, PubMed:29438978).
Plays a role in maintaining mitochondrial homeostasis by regulating the processing of mitochondrial transcripts (PubMed:28794432).
Regulates also mRNA stability by recruiting CNOT7/CAF1 deadenylase on mRNA 3'UTR leading to poly(A) tail deadenylation and thus shortening (PubMed:30520513).
In response to oxidative insult, associates with stalled ribosomes localized to stress granules (SGs) and contributes to cell survival (PubMed:19765185, PubMed:23398327).
Participates also in the normal skeletal muscle formation and regeneration, forming cytoplasmic myo-granules and binding mRNAs that encode sarcomeric proteins (PubMed:30464263).
Plays a role in the maintenance of the circadian clock periodicity via stabilization of the CRY1 and CRY2 proteins in a FBXL3-dependent manner (PubMed:27123980).
Negatively regulates the expression of CDK6 (PubMed:19760257).
Regulates the expression of HDAC6, ATG7 and VCP in a PPIA/CYPA-dependent manner (PubMed:25678563).