MOGS
适用: 人
WB, IF (p), IF (cc)
宿主: 兔
Polyclonal
AbBy Fluor® 647
应用备注
ELISA: 1/1,000. Western Blot: 1/100-1/500. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
限制
仅限研究用
状态
Liquid
浓度
0.25 mg/mL
缓冲液
PBS with 0.09 % (W/V) Sodium Azide as preservative.
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 the antibody undiluted at 2-8 °C for one month or (in aliquots) at-20 °C for longer.
GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.Synonyms: Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I