產品詳情
簡單介紹：
Anti-AHI1抗體產品質量穩定，實驗效果明顯，貨期快，價格優惠，歡迎垂詢訂購！我公司長期供應**組化抗體、WB抗體、**組化試劑盒和抗體試驗所需全部相關試劑、熒光標記抗體、單克隆抗體、多克隆抗體、各種標記的二抗IgG/IgM/IgD/IgA等科研實驗抗體。Anti-AHI1抗體用于**組化實驗,WB實驗,相應的標記抗體有HRP標記抗體,FITC標記,BIO等。
詳情介紹：
Rabbit Anti-AHI1
Cat. Number:
Anti-AHI1抗體KL-7854R
Quantity size:
1mg dilute with 1ml 0.01 M PBS(pH7.4) or antibody diluent.
Background:
Highly expressed in Anti-AHI1抗體the most primitive normal hematopoietic cells. Expressed in brain, particularly in neurons that give rise to the crossing axons of the corticospinal tract and superior cerebellar peduncles. Expressed in kidney (renal collecting duct cells) (at protein level).
Involvement in disease:Defects in AHI1 are the cause of Joubert syndrome type 3 (JBTS3) . JBTS is an autosomal recessive disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease. JBTS3 shows minimal Anti-AHI1抗體extra central nervous system involvement and appears not to be associated with renal dysfunction.
Involvement in disease:Defects in AHI1 are the cause of Joubert syndrome type 3 (JBTS3) . JBTS is an autosomal recessive disorder presenting with cerebellar ataxia, oculomotor apraxia, hypotonia, neonatal breathing abnormalities and psychomotor delay. Neuroradiologically, it is characterized by cerebellar vermian hypoplasia/aplasia, thickened and reoriented superior cerebellar peduncles, and an abnormally large interpeduncular fossa, giving the appearance of a molar tooth on transaxial slices (molar tooth sign). Additional variable features include retinal dystrophy and renal disease. JBTS3 shows minimal Anti-AHI1抗體extra central nervous system involvement and appears not to be associated with renal dysfunction.
Also known as:
Abelson helper integration Anti-AHI1抗體site 1 protein homolog; Abelson helper integration site 1; Abelson helper integration site; AHI 1; AHI-1; Ahi1; AHI1_HUMAN; Contatins SH3 and WD40 domains; JBTS3; Jouberin; ORF1.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, .
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Immunogen: KLH conjugated synthetic peptide derived from human AHI1.
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Predicted Molecular Weight: 137kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions Anti-AHI1抗體must be determined by the end user.
Not yet tested in other applications.
Optimal working dilutions Anti-AHI1抗體must be determined by the end user.
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