Anti-SUMF1 Antibody (CAB14304)
- SKU:
- CAB14304
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-SUMF1 Antibody |
抗体コード: | CAB14304 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB |
反応性: | Human |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 34-374 of human SUMF1 (NP_877437.2). |
申し込み: | WB |
推奨希釈: | WB 1:500 - 1:2000 |
反応性: | Human |
ポジティブサンプル: | HepG2, Jurkat |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 34-374 of human SUMF1 (NP_877437.2). |
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: | SQEA GTGA GAGS LAGS CGCG TPQR PGAH GSSA AAHR YSRE ANAP GPVP GERQ LAHS KMVP IPAG VFTM GTDD PQIK QDGE APAR RVTI DAFY MDAY EVSN TEFE KFVN STGY LTEA EKFG DSFV FEGM LSEQ VKTN IQQA VAAA PWWL PVKG ANWR HPEG PDST ILHR PDHP VLHV SWND AVAY CTWA GKRL PTEA EWEY SCRG GLHN RLFP WGNK LQPK GQHY ANIW QGEF PVTN TGED GFQG TAPV DAFP PNGY GLYN IVGN AWEW TSDW WTVH HSVE ETLN PKGP PSGK DRVK KGGS YMCH RSYC YRYR CAAR SQNT PDSS ASNL GFRC AADR LPTM D |
遺伝子ID: | 285362 |
Uniprot: | Q8NBK3 |
セルラーロケーション: | Endoplasmic reticulum lumen |
計算された分子量: | 31kDa/37kDa/38kDa/40kDa/46kDa |
観察された分子量: | 41kDa |
同義語: | SUMF1, AAPA3037, FGE, UNQ3037 |
バックグラウンド: | This gene encodes an enzyme that catalyzes the hydrolysis of sulfate esters by oxidizing a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also known as C-alpha-formylglycine. Mutations in this gene cause multiple sulfatase deficiency, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants. |
UniProt Protein Function: | SUMF1: Using molecular oxygen and an unidentified reducing agent, oxidizes a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also called C(alpha)-formylglycine. Known substrates include GALNS, ARSA, STS and ARSE. 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. Belongs to the sulfatase-modifying factor family. 3 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Secreted, signal peptide; EC 1.8.99.-; Endoplasmic reticulum; Secreted; Oxidoreductase Chromosomal Location of Human Ortholog: 3p26.1 Cellular Component: endoplasmic reticulum lumen Molecular Function:oxidoreductase activity Biological Process: glycosphingolipid metabolic process; post-translational protein modification Disease: Multiple Sulfatase Deficiency |
NCBI Summary: | This gene encodes an enzyme that catalyzes the hydrolysis of sulfate esters by oxidizing a cysteine residue in the substrate sulfatase to an active site 3-oxoalanine residue, which is also known as C-alpha-formylglycine. Mutations in this gene cause multiple sulfatase deficiency, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2009] |
UniProt Code: | Q8NBK3 |
NCBI GenInfo Identifier: | 62298562 |
NCBI Gene ID: | 285362 |
NCBI Accession: | Q8NBK3.3 |
UniProt Secondary Accession: | Q8NBK3,Q0VAC6, Q0VAC7, Q2NL78, Q53ZE4, Q6UY39, Q96AK5 Q96DK8, B4DXK5, B7XD05, E9PGL0, G5E9B0, |
UniProt Related Accession: | Q8NBK3 |
Molecular Weight: | 38,386 Da |
NCBI Full Name: | Sulfatase-modifying factor 1 |
NCBI Synonym Full Names: | sulfatase modifying factor 1 |
NCBI Official Symbol: | SUMF1 |
NCBI Official Synonym Symbols: | FGE; UNQ3037; AAPA3037 |
NCBI Protein Information: | sulfatase-modifying factor 1 |
UniProt Protein Name: | Sulfatase-modifying factor 1 |
UniProt Synonym Protein Names: | C-alpha-formylglycine-generating enzyme 1 |
Protein Family: | Sulfatase-modifying factor |
UniProt Gene Name: | SUMF1 |
UniProt Entry Name: | SUMF1_HUMAN |
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