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海藻酸鈣號：9005-35-0   
英文名稱：Alginic Acid Calcium Salt；Calcium Alginate   
其他名稱：藻酸鈣；褐藻酸鈣   
號：9005-35-0   
級別：BR   
干燥失重：≤20%   
性狀(以下信息僅供參考)：粉末。   
用途：本品僅供科研，不得用于其它用途   
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研究領域  神經(jīng)生物學 信號轉導 細胞骨架 細胞外基質 
蛋白分子量  predicted molecular weight: 55kDa
性    狀  Lyophilized or Liquid
免 疫 原  KLH conjugated synthetic peptide derived from human Sulphamidase (301-388aa)
亞    型  IgG
純化方法  affinity purified by Protein A
儲 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
產(chǎn)品應用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500
（石蠟切片需做抗原修復）
 not yet tested in other applications.
 optimal dilutions/concentrations should be determined by the end user. 
保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.
Subcellular Location : Lysosome.
Post-translational modifications : The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity.
DISEASE : Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A) [MIM:252900]; also known as Sanfilippo syndrome A. MPS3A is a severe form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years; severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life. MPS3A is characterized by earlier onset, rapid progression of symptoms and shorter survival.
Similarity : Belongs to the sulfatase family.
Database links : UniProtKB/Swiss-Prot: P51688.1
英文名稱  Anti-SMUBP2
中文名稱  免疫球蛋白μ鏈結合蛋白2抗體
別    名  AEP; Antifreeze enhancer binding protein; ATP-dependent helicase IGHMBP2; Cardiac transcription factor 1; Cardiac transcription factor1; CATF 1; CATF1; DNA-binding protein SMUBP-2; GF-1; Glial factor 1; HCSA; HMN 6; HMN6; IGHMBP 2; Ighmbp2; Immunoglobulin mu binding protein 2; Immunoglobulin mu binding protein2; Immunoglobulin mu-binding protein 2; Immunoglobulin S mu binding protein 2; Immunoglobulin S mu binding protein2; RIPE3 b1; RIPE3b 1; RIPE3b1; SMARD 1; SMARD1; SMBP2_HUMAN; SMUBP 2.
濃    度  1mg/1ml
規(guī) 格  0.2ml/200μg
抗體來源  Rabbit 
克隆類型  polyclonal
交叉反應  Human, Mouse, Rat, Pig, Cow, Sheep 
產(chǎn)品類型  一抗   
研究領域  心血管 細胞生物 神經(jīng)生物學 信號轉導 表觀遺傳學 
蛋白分子量  predicted molecular weight: 109kDa
性    狀  Lyophilized or Liquid
免 疫 原  KLH conjugated synthetic peptide derived from human SMUBP2 (271-355aa)
亞    型  IgG
純化方法  affinity purified by Protein A
儲 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
產(chǎn)品應用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500
（石蠟切片需做抗原修復）
 not yet tested in other applications.
 optimal dilutions/concentrations should be determined by the end user. 
保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 IGHMBP2 is a 993 amino acid nuclear and cytoplasmic protein that is ubiquitously expressed. Belonging to the DNA2/NAM7 helicase family, IGHMBP2 is a 5' to 3' helicase that unwinds RNA and DNA duplexes in an ATP-dependent reaction. IGHMBP2 also acts as a transcriptional regulator and is necessary for transcriptional activation of the flounder liver-type antifreeze protein gene. IGHMBP2 exists as a homooligomer and is part of the cytosolic ribonucleoprotein complex. Mutations in the gene encoding IGHMBP2 are suggested to lead to distal hereditary motor neuronopathy type 6 (HMN6), also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). HMN6 is characterized by weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs and severe respiratory distress.
Function : 5' to 3' helicase that unwinds RNA and DNA duplices in an ATP-dependent reaction. Acts as a transcription regulator. Required for the transcriptional activation of the flounder liver-type antifreeze protein gene. Exhibits strong binding specificity to the enhancer element B of the flounder antifreeze protein gene intron. Binds to the insulin II gene RIPE3B enhancer region. May be involved in translation (By similarity). DNA-binding protein specific to 5'-phosphorylated single-stranded guanine-rich sequence related to the immunoglobulin mu chain switch region. Preferentially binds to the 5'-GGGCT-3' motif. Interacts with tRNA-Tyr. Stimulates the transcription of the human neurotropic virus JCV.
Subunit : Homooligomer. Interacts with RUVBL1, RUVBL2, GTF3C1 andABT1. Is part of large cytosolic ribonucleoprotein complexes(Probable). Associates with the ribosomes.
Subcellular Location : Nucleus. Cytoplasm.
Tissue Specificity : Expressed in all tissues examined.
Post-translational modifications : Phosphorylated upon DNA damage, probably by ATM or ATR.
DISEASE : Defects in IGHMBP2 are the cause of distal hereditary motor neuronopathy type 6 (HMN6) [MIM:604320]; also known as spinal muscular atrophy distal autosomal recessive 1 (DSMA1) or spinal muscular atrophy with respiratory distress 1 (SMARD1). Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective degeneration of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs. The most prominent symptoms of HMN6 are severe respiratory distress resulting from diaphragmatic paralysis with eventration shown on chest x-ray and predominant involvement of the upper limbs and distal muscles.
Similarity : Belongs to the DNA2/NAM7 helicase family.
Contains 1 AN1-type zinc finger.
Contains 1 R3H domain.
Database links : UniProtKB/Swiss-Prot: P38935.3
英文名稱  Anti-SPG21