SUMF1 抗体 (Internal Region)
Our Local Distributor
北京 101111
Quick Overview for SUMF1 抗体 (Internal Region) (ABIN401525)
抗原
See all SUMF1 抗体适用
宿主
克隆类型
标记
应用范围
-
-
抗原表位
- Internal Region
-
序列
- c-ETLNPKGPPS GKDR
-
特异性
- This antibody detects SUMF1 / FGE (Internal).
-
交叉反应 (详细)
- Species reactivity (expected):Rat, Dog, CowSpecies reactivity (tested):Human, Mouse
-
纯化方法
- Ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide
-
免疫原
- Peptide from the internal region of the protein sequence according to NP_877437.2 . Genename: SUMF1
-
-
-
-
应用备注
-
Peptide ELISA: antibody detection limit dilution 1: 16000. Western blot: 0.5-2 μg/mL. Approx 37-40 kDa band observed in Mouse Pancreas, Mouse Eyeand Human Kidney lysates (calculated MW of 40.0 kDa according to NP_877437.2).
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
限制
- 仅限研究用
-
-
-
浓度
- 0.5 mg/mL
-
缓冲液
- Tris saline, 0.02 % sodium azide, pH 7.3 with 0.5 % bovine serum albumin
-
储存液
- Sodium azide
-
注意事项
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
-
注意事项
- Avoid repeated freezing and thawing.
-
储存条件
- 4 °C/-20 °C
-
储存方法
- Store at 2 - 8 °C for up to one month or (in aliquots) at -20 °C.
-
-
- SUMF1 (Sulfatase Modifying Factor 1 (SUMF1))
-
别名
- SUMF1 / FGE
-
背景
- SUMF1 is a sulfatase modifying enzyme that oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Defects in SUMF1 are the cause of multiple sulfatase deficiency (MSD). MSD is a clinically and biochemically heterogeneous disorder caused by the simultaneous impairment of all sulfatases, due to defective post-translational modification and activation. It combines features of individual sulfatase deficiencies such as metachromatic leukodystrophy, mucopolysaccharidosis, chondrodysplasia punctata, hydrocephalus, ichthyosis, neurologic deterioration and developmental delay. Inheritance is autosomal recessive.Synonyms: C-alpha-formylglycine-generating enzyme 1, Sulfatase-modifying factor 1
-
基因ID
- 285362
-
NCBI登录号
- NP_877437
-
UniProt
- Q8NBK3
抗原
-