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中文名稱  泛酸激酶2抗體規(guī)格 

英文名稱  Anti-PANK2

別    名 C20orf48; HARP; hPANK2; HSS; MGC15053; NBIA1; PANK2; PANK2_HUMAN; Pantothenate kinase 2 (Hallervorden Spatz syndrome); Pantothenate kinase 2; Pantothenic acid kinase 2; PKAN; RP23 387C21.4.

產(chǎn)品屬性：

泛酸激酶2抗體規(guī)格 濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Dog, Pig, Cow, Horse

產(chǎn)品類型   一抗  

研究領(lǐng)域     細胞生物 免疫學(xué) 神經(jīng)生物學(xué) 信號轉(zhuǎn)導(dǎo)

蛋白分子量  predicted molecular weight: 57kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human PANK2

亞    型  IgG

純化方法   affinity purified by Protein A

儲 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

泛酸激酶2抗體規(guī)格 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1); also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.

Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.

Function : May be the master regulator of the CoA biosynthesis (By similarity).

Subunit :

Isoform 2: Cytoplasm (Potential).

Tissue Specificity : Ubiquitous.

DISEASE : Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1) [MIM:234200]; also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.

Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP) [MIM:607236]. HARP is a rare syndrome with many clinical similarities to NBIA1.

Similarity : Belongs to the type II pantothenate kinase family.

Database links : UniProtKB/Swiss-Prot: Q9BZ23.3

實驗流程：

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泛酸激酶2抗體規(guī)格 全：公司提供上萬種產(chǎn)品，涵蓋了生物試劑，elisa試劑盒，標(biāo)準(zhǔn)品，培養(yǎng)基，原裝耗材，抗體、培養(yǎng)基、ATCC細胞等，基本上各種科研所需產(chǎn)品在我司都能找到。

新：產(chǎn)品更新速度較快，基本上每周都有新產(chǎn)品出現(xiàn)。

優(yōu)：產(chǎn)品質(zhì)量好，投訴比較少。

好：我公司具有優(yōu)質(zhì)的技術(shù)團隊，產(chǎn)品一旦售出，實驗過程中遇到困難可提供在線技術(shù)咨詢。使您使用產(chǎn)品時沒有任何的后顧之憂。

技術(shù)外包服務(wù):

★泛酸激酶2抗體規(guī)格 分子生物學(xué)：質(zhì)粒抽提、PCR、Q-PCR、RT-PCR、分子生物學(xué)：基因合成、引物合成、基因測序、載體構(gòu)建等

★蛋白工程：原核、哺乳動物蛋白表達系統(tǒng)等

★病毒包裝：腺病毒、慢病毒等

★抗體工程：磁珠分選、病理染色、WB、ELISA、IP、IF、IHC、FACS、Confocal等等

★細胞工程：細胞表型分析(凋亡、增殖、周期、遷移、侵襲、修復(fù)、克隆形成)、細胞培養(yǎng)、細胞膜制備、穩(wěn)定細胞株構(gòu)建、細胞RNAi技術(shù)等等。

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