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FHL1 rabbit pAb
商品貨號： PLA021221
適 應(yīng) 性： 人,小鼠,大鼠
WB
￥600元
規(guī)格：
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MSDS
說明書
商品描述
  • 基因名稱： FHL1 SLIM1
  • 蛋白名稱： FHL1
  • Human_gene_id： 2273
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2273
  • Human_swiss_prot_no： Q13642
  • Human_swiss_link： https://www.uniprot.org/uniprotkb/Q13642/entry
  • Mouse_gene_id： 14199
  • Mouse_gene_link： https://www.uniprot.org/uniprot/14199
  • Mouse_swiss_prot_no： P97447
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P97447
  • Rat_gene_id： 25177
  • Rat_gene_link： https://www.uniprot.org/uniprot/25177
  • Rat_swiss_prot_no： Q9WUH4
  • Rat_swiss_link： https://www.uniprot.org/uniprotkb/Q9WUH4
  • 特異性： This antibody detects endogenous levels of FHL1 at Human/Mouse/Rat
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1：500-2000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說明書： 1
  • Msds： MSDS_Antibody.pdf
  • 分子量： 36kD
  • 功能： developmental stage:Elevated levels during postnatal muscle growth.,disease:Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717].,disease:Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear.,disease:Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies.,disease:Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases.,function:May have an involvement in muscle development or hypertrophy.,similarity:Contains 2 LIM zinc-binding domains.,similarity:Contains 3 LIM zinc-binding domains.,similarity:Contains 4 LIM zinc-binding domains.,subcellular location:Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.,tissue specificity:Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.,
  • 相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
  • 細胞定位： [Isoform 1]: Cytoplasm.; [Isoform 3]: Cytoplasm. Nucleus.; [Isoform 2]: Nucleus. Cytoplasm, cytosol. Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.
  • 組織表達： Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.
  • 科研貨號： PLA021221
FHL1 rabbit pAb
Catalog No PLA021221
Product information
  • 基因名稱： FHL1 SLIM1
  • 蛋白名稱： FHL1
  • Human_gene_id： 2273
  • Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=2273
  • Human_swiss_prot_no： Q13642
  • Human_swiss_link： https://www.uniprot.org/uniprotkb/Q13642/entry
  • Mouse_gene_id： 14199
  • Mouse_gene_link： https://www.uniprot.org/uniprot/14199
  • Mouse_swiss_prot_no： P97447
  • Mouse_swiss_link： https://www.uniprot.org/uniprotkb/P97447
  • Rat_gene_id： 25177
  • Rat_gene_link： https://www.uniprot.org/uniprot/25177
  • Rat_swiss_prot_no： Q9WUH4
  • Rat_swiss_link： https://www.uniprot.org/uniprotkb/Q9WUH4
  • 特異性： This antibody detects endogenous levels of FHL1 at Human/Mouse/Rat
  • 組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 來源： Polyclonal, Rabbit,IgG
  • 稀釋： WB 1：500-2000
  • 純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度： 1 mg/ml
  • 儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
  • 說明書： 1
  • Msds： MSDS_Antibody.pdf
  • 分子量： 36kD
  • 功能： developmental stage:Elevated levels during postnatal muscle growth.,disease:Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717].,disease:Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear.,disease:Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies.,disease:Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases.,function:May have an involvement in muscle development or hypertrophy.,similarity:Contains 2 LIM zinc-binding domains.,similarity:Contains 3 LIM zinc-binding domains.,similarity:Contains 4 LIM zinc-binding domains.,subcellular location:Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.,tissue specificity:Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.,
  • 相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
  • 細胞定位： [Isoform 1]: Cytoplasm.; [Isoform 3]: Cytoplasm. Nucleus.; [Isoform 2]: Nucleus. Cytoplasm, cytosol. Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.
  • 組織表達： Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.
  • 科研貨號： PLA021221
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