Osenses

CLCN7 Polyclonal Antibody

产品信息

OSC00248W-100UL

应用

建议稀释比

已发表文章

免疫印迹 (WB)

1:300-1:2,000

免疫组化 (IHC)

1:300-1:2,000

其他PubMed (Misc)

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产品规格

种属反应

Human, Mouse, Rat

已发表种属

Not Applicable

宿主/亚型

Rabbit / Ig

分类

Polyclonal

类型

Antibody

抗原

A synthetic peptide from n-terminal region of human Chloride channel protein 7 (CLCN7) conjugated to an immunogenic carrier protein was used as the antigen

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偶联物

Unconjugated Unconjugated Unconjugated

形式

Lyophilized

浓度

Conc. Not Determined

保存液

whole serum

内含物

no preservative

保存条件

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. Glycerol (1:1) may be added for added stability.

运输条件

Ambient (domestic); Wet ice (international)

产品详细信息

Reconstitute in 100 µL of sterile water. Centrifuge to remove any insoluble material.

Specificity of this antibody: CLCN7.

靶标信息

The product of this gene belongs to the CLC chloride channel family of proteins. Chloride channels play important roles in the plasma membrane and in intracellular organelles. This gene encodes chloride channel 7. Defects in this gene are the cause of osteopetrosis autosomal recessive type 4 (OPTB4), also called infantile malignant osteopetrosis type 2 as well as the cause of autosomal dominant osteopetrosis type 2 (OPTA2), also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood.

仅用于科研。不用于诊断过程。未经明确授权不得转售。

Product References

DNA-based diagnosis of malignant osteopetrosis by whole-genome scan using a single-nucleotide polymorphism microarray: standardization of molecular investigations of genetic diseases due to consanguinity.

Journal of human genetics

Lam CW,Tong SF,Wong K,Luo YF,Tang HY,Ha SY,Chan MH

Fri Mar 16 00:00:00 UTC 2007

Global, in vivo, and site-specific phosphorylation dynamics in signaling networks.

Cell

Olsen JV,Blagoev B,Gnad F,Macek B,Kumar C,Mortensen P,Mann M

Fri Nov 03 00:00:00 UTC 2006

The sequence and analysis of duplication-rich human chromosome 16.

Nature

Martin J,Han C,Gordon LA,Terry A,Prabhakar S,She X,Xie G,Hellsten U,Chan YM,Altherr M,Couronne O,Aerts A,Bajorek E,Black S,Blumer H,Branscomb E,Brown NC,Bruno WJ,Buckingham JM,Callen DF,Campbell CS,Campbell ML,Campbell EW,Caoile C,Challacombe JF,Chasteen LA,Chertkov O,Chi HC,Christensen M,Clark LM,Cohn JD,Denys M,Detter JC,Dickson M,Dimitrijevic-Bussod M,Escobar J,Fawcett JJ,Flowers D,Fotopulos D,Glavina T,Gomez M,Gonzales E,Goodstein D,Goodwin LA,Grady DL,Grigoriev I,Groza M,Hammon N,Hawkins T,Haydu L,Hildebrand CE,Huang W,Israni S,Jett J,Jewett PB,Kadner K,Kimball H,Kobayashi A,Krawczyk MC,Leyba T,Longmire JL,Lopez F,Lou Y,Lowry S,Ludeman T,Manohar CF,Mark GA,McMurray KL,Meincke LJ,Morgan J,Moyzis RK,Mundt MO,Munk AC,Nandkeshwar RD,Pitluck S,Pollard M,Predki P,Parson-Quintana B,Ramirez L,Rash S,Retterer J,Ricke DO,Robinson DL,Rodriguez A,Salamov A,Saunders EH,Scott D,Shough T,Stallings RL,Stalvey M,Sutherland RD,Tapia R,Tesmer JG,Thayer N,Thompson LS,Tice H,Torney DC,Tran-Gyamfi M,Tsai M,Ulanovsky LE,Ustaszewska A,Vo N,White PS,Williams AL,Wills PL,Wu JR,Wu K,Yang J,Dejong P,Bruce D,Doggett NA,Deaven L,Schmutz J,Grimwood J,Richardson P,Rokhsar DS,Eichler EE,Gilna P,Lucas SM,Myers RM,Rubin EM,Pennacchio LA

Thu Dec 23 00:00:00 UTC 2004

Complete sequencing and characterization of 21,243 full-length human cDNAs.

Nature genetics

Ota T,Suzuki Y,Nishikawa T,Otsuki T,Sugiyama T,Irie R,Wakamatsu A,Hayashi K,Sato H,Nagai K,Kimura K,Makita H,Sekine M,Obayashi M,Nishi T,Shibahara T,Tanaka T,Ishii S,Yamamoto J,Saito K,Kawai Y,Isono Y,Nakamura Y,Nagahari K,Murakami K,Yasuda T,Iwayanagi T,Wagatsuma M,Shiratori A,Sudo H,Hosoiri T,Kaku Y,Kodaira H,Kondo H,Sugawara M,Takahashi M,Kanda K,Yokoi T,Furuya T,Kikkawa E,Omura Y,Abe K,Kamihara K,Katsuta N,Sato K,Tanikawa M,Yamazaki M,Ninomiya K,Ishibashi T,Yamashita H,Murakawa K,Fujimori K,Tanai H,Kimata M,Watanabe M,Hiraoka S,Chiba Y,Ishida S,Ono Y,Takiguchi S,Watanabe S,Yosida M,Hotuta T,Kusano J,Kanehori K,Takahashi-Fujii A,Hara H,Tanase TO,Nomura Y,Togiya S,Komai F,Hara R,Takeuchi K,Arita M,Imose N,Musashino K,Yuuki H,Oshima A,Sasaki N,Aotsuka S,Yoshikawa Y,Matsunawa H,Ichihara T,Shiohata N,Sano S,Moriya S,Momiyama H,Satoh N,Takami S,Terashima Y,Suzuki O,Nakagawa S,Senoh A,Mizoguchi H,Goto Y,Shimizu F,Wakebe H,Hishigaki H,Watanabe T,Sugiyama A,Takemoto M,Kawakami B,Yamazaki M,Watanabe K,Kumagai A,Itakura S,Fukuzumi Y,Fujimori Y,Komiyama M,Tashiro H,Tanigami A,Fujiwara T,Ono T,Yamada K,Fujii Y,Ozaki K,Hirao M,Ohmori Y,Kawabata A,Hikiji T,Kobatake N,Inagaki H,Ikema Y,Okamoto S,Okitani R,Kawakami T,Noguchi S,Itoh T,Shigeta K,Senba T,Matsumura K,Nakajima Y,Mizuno T,Morinaga M,Sasaki M,Togashi T,Oyama M,Hata H,Watanabe M,Komatsu T,Mizushima-Sugano J,Satoh T,Shirai Y,Takahashi Y,Nakagawa K,Okumura K,Nagase T,Nomura N,Kikuchi H,Masuho Y,Yamashita R,Nakai K,Yada T,Nakamura Y,Ohara O,Isogai T,Sugano S

Thu Jan 01 00:00:00 UTC 2004

Chloride channel ClCN7 mutations are responsible for severe recessive, dominant, and intermediate osteopetrosis.

Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research

Frattini A,Pangrazio A,Susani L,Sobacchi C,Mirolo M,Abinun M,Andolina M,Flanagan A,Horwitz EM,Mihci E,Notarangelo LD,Ramenghi U,Teti A,Van Hove J,Vujic D,Young T,Albertini A,Orchard PJ,Vezzoni P,Villa A

Wed Oct 01 00:00:00 UTC 2003

生物信息学

蛋白别名: Chloride channel 7 alpha subunit; Chloride channel protein 7; chloride channel, voltage-sensitive 7; ClC-7; CLCN-7; H(+)/Cl(-) exchange transporter 7; protein phosphatase 1, regulatory subunit 63

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基因别名: AA409691; AW538136; CLC-7; CLC7; CLCN7; D17Wsu51e; OPTA2; OPTB4; PPP1R63

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UniProt ID: (Human) P51798, (Rat) P51799, (Mouse) O70496

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Entrez Gene ID: (Human) 1186, (Rat) 29233, (Mouse) 26373

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功能