Anti-IDS Antibody (CAB1857)
- SKU:
- CAB1857
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-IDS Antibody |
抗体コード: | CAB1857 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 34-290 of human IDS (NP_006114.1). |
申し込み: | WB IHC |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | HepG2, A-549, HT-1080, Mouse kidney, Rat kidney |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 34-290 of human IDS (NP_006114.1). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | TDAL NVLL IIVD DLRP SLGC YGDK LVRS PNID QLAS HSLL FQNA FAQQ AVCA PSRV SFLT GRRP DTTR LYDF NSYW RVHA GNFS TIPQ YFKE NGYV TMSV GKVF HPGI SSNH TDDS PYSW SFPP YHPS SEKY ENTK TCRG PDGE LHAN LLCP VDVL DVPE GTLP DKQS TEQA IQLL EKMK TSAS PFFL AVGY HKPH IPFR YPKE FQKL YPLE NITL APDP EVPD GLPP VAYN PWMD IRQR EDVQ ALNI SVPY GPIP V |
遺伝子ID: | 3423 |
Uniprot: | P22304 |
セルラーロケーション: | Lysosome |
計算された分子量: | 34kDa/38kDa/61kDa |
観察された分子量: | 76kDa |
同義語: | IDS, MPS2, SIDS |
バックグラウンド: | This gene encodes a member of the sulfatase family of proteins. The encoded preproprotein is proteolytically processed to generate two polypeptide chains. This enzyme is involved in the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease mucopolysaccharidosis type II, also known as Hunter syndrome. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. |
UniProt Protein Function: | IDS: Required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2); also known as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized by intracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine. Most children with MPS2 have a severe form with early somatic abnormalities including skeletal deformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominent feature is neurological damage that presents as developmental delay and hyperactivity but progresses to mental retardation and dementia. They die before 15 years of age, usually as a result of obstructive airway disease or cardiac failure. In contrast, those with a mild form of MPS2 may survive into adulthood, with attenuated somatic complications and often without mental retardation. Belongs to the sulfatase family. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Hydrolase; EC 3.1.6.13; Glycan Metabolism - glycosaminoglycan degradation Chromosomal Location of Human Ortholog: Xq28 Cellular Component: lysosomal lumen Molecular Function:metal ion binding; iduronate-2-sulfatase activity Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan catabolic process; glycosaminoglycan metabolic process; carbohydrate metabolic process; chondroitin sulfate catabolic process; pathogenesis Disease: Mucopolysaccharidosis, Type Ii |
NCBI Summary: | The protein encoded by this gene belongs to the sulfatase family, is localized to the lysosome, and is involved in lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this gene are associated with the X-linked lysosomal storage disease, mucopolysaccharidosis type II, also known as Hunter syndrome. Alternatively spliced transcript variants have been described for this gene. [provided by RefSeq, Aug 2013] |
UniProt Code: | P22304 |
NCBI GenInfo Identifier: | 124174 |
NCBI Gene ID: | 3423 |
NCBI Accession: | P22304.1 |
UniProt Secondary Accession: | P22304,Q14604, Q9BRM3, D3DWT4, |
UniProt Related Accession: | P22304 |
Molecular Weight: | 34,893 Da |
NCBI Full Name: | Iduronate 2-sulfatase |
NCBI Synonym Full Names: | iduronate 2-sulfatase |
NCBI Official Symbol: | IDS |
NCBI Official Synonym Symbols: | MPS2; SIDS |
NCBI Protein Information: | iduronate 2-sulfatase; idursulfase; iduronate 2-sulfatase 14 kDa chain; iduronate 2-sulfatase 42 kDa chain; alpha-L-iduronate sulfate sulfatase |
UniProt Protein Name: | Iduronate 2-sulfatase |
UniProt Synonym Protein Names: | Alpha-L-iduronate sulfate sulfatase; Idursulfase |
Protein Family: | Iduronate 2-sulfatase |
UniProt Gene Name: | IDS |
UniProt Entry Name: | IDS_HUMAN |
View AllClose