FH 抗体 (Biotin)
Our Local Distributor
北京 101111
Quick Overview for FH 抗体 (Biotin) (ABIN568201)
抗原
See all FH 抗体适用
宿主
克隆类型
标记
应用范围
-
-
产品特性
- Molar Ratio: Biotin/IgG ~7.6
-
纯化方法
- Ammonium Sulphate Precipitation and Ion Exchange Chromatography
-
免疫原
- Fumarase is isolated and purified from Porcine heart Freund’s complete adjuvant is used in the first step of the immunization procedure.
-
亚型
- IgG
-
-
-
-
应用备注
- Optimal working dilution should be determined by the investigator.
-
限制
- 仅限研究用
-
-
-
状态
- Liquid
-
溶解方式
- Restore by adding 1.0 mL of sterile distilled water
-
浓度
- 10.0 mg/mL
-
缓冲液
- PBS, pH 7.2 without preservatives and foreign proteins
-
储存液
- Without preservative
-
储存条件
- 4 °C/-20 °C
-
储存方法
- Store the antibody lyophilized at 2-8 °C and reconstituted at 2-8 °C for one week or (in aliquots) at -20 °C for longer. If a slight precipitation occurs upon storage, this should be removed by centrifugation.
-
-
- FH (Fumarate Hydratase (FH))
-
别名
- Fumarase
-
背景
- Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812], also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids). Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].Synonyms: HLRCC, LRCC, MCL, MCUL1, mitochondrial Fumarate hydratase
-
基因ID
- 9823
-
UniProt
- P10173
抗原
-