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Rabbit Anti-Hemoglobin subunit gamma 2/BF488 Conjugated antibody (bs-16469R-BF488)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-16469R-BF488
英文名稱 Rabbit Anti-Hemoglobin subunit gamma 2/BF488 Conjugated antibody
中文名稱 BF488標記的血紅蛋白γ2抗體
別    名 Abnormal hemoglobin; FLJ76540; G gamma globin; Gamma 2 globin; Gamma-2-globin; Hb F Ggamma; HBG 2; HBG2; HBG2_HUMAN; Hemoglobin gamma 2 chain; Hemoglobin gamma G; Hemoglobin gamma G chain; Hemoglobin gamma-2 chain; Hemoglobin gamma-G chain; Hemoglobin subunit gamma 2; Hemoglobin subunit gamma-2; Methemoglobin; OTTHUMP00000069638.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, )
產(chǎn)品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 16kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Hemoglobin subunit gamma 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
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.
產(chǎn)品介紹 background:
The gamma globin genes (HBG1 and HBG2) are normally expressed in the fetal liver, spleen and bone marrow. Two gamma chains together with two alpha chains constitute fetal hemoglobin (HbF) which is normally replaced by adult hemoglobin (HbA) at birth. In some beta-thalassemias and related conditions, gamma chain production continues into adulthood. The two types of gamma chains differ at residue 136 where glycine is found in the G-gamma product (HBG2) and alanine is found in the A-gamma product (HBG1). The former is predominant at birth. The order of the genes in the beta-globin cluster is: 5'- epsilon -- gamma-G -- gamma-A -- delta -- beta--3'. [provided by RefSeq, Jul 2008]

Function:
Gamma chains make up the fetal hemoglobin F, in combination with alpha chains.

Subcellular Location:
Belongs to the globin family.

Tissue Specificity:
Red blood cells.

Post-translational modifications:
Acetylation of Gly-2 converts Hb F to the minor Hb F1.

DISEASE:
Defects in HBG2 are the cause of cyanosis transient neonatal (TNCY) [MIM:613977]. TNCY is a disorder characterized by cyanosis in the fetus and neonate, due to a defect in the fetal hemoglobin chain which has reduced affinity for oxygen. Some patients develop anemia resulting from increased destruction of red cells containing abnormal or unstable hemoglobin. The cyanosis resolves spontaneously by 5 to 6 months of age or earlier, as the adult beta-globin chain is produced and replaces the fetal gamma-globin chain.

Similarity:
Belongs to the globin family.

Database links:

Entrez Gene: 3048 Human

Omim: 142250 Human

SwissProt: P69892 Human

Unigene: 302145 Human

Unigene: 712539 Human



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