FUCA1 抗体 (N-Term)
Quick Overview for FUCA1 抗体 (N-Term) (ABIN2785639)
抗原
See all FUCA1 抗体适用
宿主
克隆类型
标记
应用范围
-
-
抗原表位
- N-Term
-
序列
- PSPVSWNWNS KDVGPHRDLV GELGTALRKR NIRYGLYHSL LEWFHPLYLL
-
预测反应
- Cow: 100%, Dog: 93%, Guinea Pig: 93%, Horse: 93%, Human: 100%, Mouse: 100%, Pig: 100%, Rabbit: 79%, Rat: 100%
-
产品特性
- This is a rabbit polyclonal antibody against FUCA1. It was validated on Western Blot using a cell lysate as a positive control.
-
纯化方法
- Affinity Purified
-
免疫原
- The immunogen is a synthetic peptide directed towards the N terminal region of human FUCA1
-
-
-
-
应用备注
- Optimal working dilutions should be determined experimentally by the investigator.
-
说明
-
Antigen size: 466 AA
-
限制
- 仅限研究用
-
-
-
状态
- Liquid
-
浓度
- Lot specific
-
缓冲液
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
-
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 freeze-thaw cycles.
-
储存条件
- -20 °C
-
储存方法
- For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
-
-
- FUCA1 (Fucosidase, alpha-L- 1, Tissue (FUCA1))
-
别名
- FUCA1
-
背景
-
Alpha-L-fucosidase (EC 3.2.1.51) is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. At least 2 separate polymorphic alpha-L-fucosidases are recognized in man: that in tissues, FUCA1, which is deficient in fucosidosis, and that in plasma, FUCA2. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form, coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form, and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form.[supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications.
Alias Symbols: FUCA
Protein Interaction Partner: PAXIP1, BARD1, APP, CUL3, VAV2, USP21, MARK2,
Protein Size: 466 -
分子量
- 54 kDa
-
基因ID
- 2517
-
NCBI登录号
- NM_000147, NP_000138
-
UniProt
- P04066
-
途径
- Glycosaminoglycan Metabolic Process
抗原
-