粘液样脂肪肉瘤蛋白FUS1抗体
规格：1mg/1ml
英文名： TLS/FUS
别名： 75 kDa DNA pairing protein; 75 kDa DNA-pairing protein; ALS6; Amyotrophic lateral sclerosis 6; FUS; FUS CHOP; Fus like protein; FUS-CHOP; FUS_HUMAN; FUS1; Fused in sarcoma; Fusion (involved in t(12;16
分子量： 53kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human TLS
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：细胞核
粘液样脂肪肉瘤蛋白FUS1抗体产品介绍：background: This gene encodes a multifunctional protein component of the heterogeneous nuclear ribonucleoprotein (hnRNP) complex. The hnRNP complex is involved in pre-mRNA splicing and the export of fully processed mRNA to the cytoplasm. This protein belongs to the FET family of RNA-binding proteins which have been implicated in cellular processes that include regulation of gene expression, maintenance of genomic integrity and mRNA/microRNA processing. Alternative splicing results in multiple transcript variants. Defects in this gene result in amyotrophic lateral sclerosis type 6. [provided by RefSeq]. Function: Binds both single-stranded and double-stranded DNA and promotes ATP-independent annealing of complementary single-stranded DNAs and D-loop formation in superhelical double-stranded DNA. May play a role in maintenance of genomic integrity. Subunit: Component of nuclear riboprotein complexes. Interacts with ILF3, TDRD3 and SF1. Interacts through its C-terminus with SFRS13A. Interacts with OTUB1 and SARNP. Subcellular Location: Nucleus. Tissue Specificity: Ubiquitous. Post-translational modifications: Arg-216 and Arg-218 are dimethylated, probably to asymmetric dimethylarginine. DISEASE: Note=A chromosomal aberration粘液样脂肪肉瘤蛋白FUS1抗体 involving FUS is found in a patient with malignant myxoid liposarcoma. Translocation t(12;16)(q13;p11) with DDIT3. Note=A chromosomal aberration involving FUS is a cause of acute myeloid leukemia (AML). Translocation t(16;21)(p11;q22) with ERG. Angiomatoid fibrous histiocytoma (AFH) [MIM:612160]: A distinct variant of malignant fibrous histiocytoma that typically occurs in children and adolescents and is manifest by nodular subcutaneous growth. Characteristic microscopic features include lobulated sheets of histiocyte-like cells intimately associated with areas of hemorrhage and cystic pseudovascular spaces, as well as a striking cuffing of inflammatory cells, mimicking a lymph node metastasis. Note=The disease may be caused by mutations affecting the gene represented in this entry. A chromosomal aberration involving FUS is found in a patient with angiomatoid fibrous histiocytoma. Translocation t(12;16)(q13;p11.2) with ATF1 generates a chimeric FUS/ATF1 protein. Amyotrophic lateral sclerosis 6 (ALS6) [MIM:608030]: 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 粘液样脂肪肉瘤蛋白FUS1抗体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. Note=The disease is caused by mutations affecting the gene represented in this entry. Tremor, hereditary essential 4 (ETM4) [MIM:614782]: A common movement disorder mainly characterized by postural tremor of the arms. Head, legs, trunk, voice, jaw, and facial muscles also may be involved. The condition can be aggravated by emotions, hunger, fatigue and temperature extremes, and may cause a functional disability or even incapacitation. Inheritance is autosomal dominant. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the RRM TET family. Contains 1 RanBP2-type zinc finger. Contains 1 RRM (RNA recognition motif) domain. Database links: Entrez Gene: 2521 Human Entrez Gene: 233908 Mouse Entrez Gene: 317385 Rat Omim: 137070 Human SwissProt: P35637 Human SwissProt: P56959 Mouse Unigene: 46894 Human Unigene: 277680 Mouse Unigene: 100218 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
粘液样脂肪肉瘤蛋白FUS1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 **学 染色质和核信号 信号转导 转录调节因子 表观遗传学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid