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Proteintech
NAGA Polyclonal Antibody
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产品信息
15096-1-AP
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
NAGA Fusion Protein Ag7172 (22-411 aa encoded by BC000095)
偶联物
形式
Liquid
浓度
0.13 mg/mL
纯化类型
Antigen affinity chromatography
保存液
PBS, pH 7.3, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Wet ice (domestic); Dry ice (international)
产品详细信息
Immunogen sequence: LLQTPPMGW LAWERFRCNI NCDEDPKNCI SEQLFMEMAD RMAQDGWRDM GYTYLNIDDC WIGGRDASGR LMPDPKRFPH GIPFLADYVH SLGLKLGIYA DMGNFTCMGY PGTTLDKVVQ DAQTFAEWKV DMLKLDGCFS TPEERAQGYP KMAAALNATG RPIAFSCSWP AYEGGLPPRV NYSLLADICN LWRNYDDIQD SWWSVLSILN WFVEHQDILQ PVAGPGHWND PDMLLIGNFG LSLEQSRAQM ALWTVLAAPL LMSTDLRTIS AQNMDILQNP LMIKINQDPL GIQGRRIHKE KSLIEVYMRP LSNKASALVF FSCRTDMPYR YHSSLGQLNF TGSVIYEAQD VYSGDIISGL RDETNFTVII NPSGVVMWYL YPIKNLEMSQ Q (22-411 aa encoded by BC000095)
靶标信息
NAGA (N-acetylgalactosaminidase, alpha), also known as alpha-galactosidase B or GALB, is a 411 lysosomal protein belonging to the glycosyl hydrolase 27 family that may exist as a homodimer and plays a critical role in glycolipid breakdown. NAGA encodes alpha-N-acetylgalactosaminidase, a lysosomal enzyme, which cleaves alpha-N-acetylgalactosaminyl groups from glycoconjugates. Mapping to human chromosome 22q13. 2, NAGA defects are the cause of an autosomal recessive disorder with three phenotypes, known as Schindler disease (types I, II and III) or NAGA deficiency (types I, II and III). Characterized by neurologic manifestations that range in severity, Schindler disease type I is the most severe form, followed by type III, which may have mild-to-moderate effects. Schindler disease type II, also known as Kanzaki disease, is characterized by mild intellectual impairment and angiokeratoma corporis diffusum.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
生物信息学
蛋白别名: Acetylgalactosaminidase, alpha-N- (alpha-galactosidase B); Alpha-galactosidase B; Alpha-N-acetylgalactosaminidase; EC 3.2.1; EC 3.2.1.49; galactosidase-alphaB; galactosidasealphaB; N-acetylgalactosaminidase, alpha-
基因别名: D22S674; GALB; NAGA
UniProt ID: (Human) P17050, (Rat) Q66H12, (Mouse) Q9QWR8
Entrez Gene ID: (Human) 4668, (Rat) 315165, (Mouse) 17939
