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Rabbit Anti-SLC25A20/AP Conjugated antibody (bs-4192R-AP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-4192R-AP
英文名稱 Rabbit Anti-SLC25A20/AP Conjugated antibody
中文名稱 堿性磷酸酶(AP)標記的線粒體二羧酸載體蛋白20抗體
別    名 CAC; CACT; Carnitine/acylcarnitine translocase; Solute carrier family 25 member 20; MCAT_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  染色質和核信號  信號轉導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, Cow, Horse, Rabbit, )
產品應用 WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 33kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SLC25A20
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
產品介紹 background:
SLC25A20 is one of several closely related mitochondrial membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. It mediates the transport of acylcarnitines into the mitochondrial matrix for their oxidation by the mitochondrial fatty acid oxidation pathway. Mutations in this gene are associated with carnitine acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.

Function:
Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.

Subcellular Location:
Mitochondrion inner membrane; Multi-pass membrane protein.

DISEASE:
Carnitine-acylcarnitine translocase deficiency (CACT deficiency) [MIM:212138]: A rare long-chain fatty acid oxidation disorder. Metabolic consequences include hypoketotic hypoglycemia under fasting conditions, hyperammonemia, elevated creatine kinase and transaminases, dicarboxylic aciduria, very low free carnitine and abnormal acylcarnitine profile with marked elevation of the long-chain acylcarnitines. Clinical features include neurologic abnormalities, cardiomyopathy, arrhythmias, skeletal muscle damage, liver dysfunction and episodes of life-threatening coma, which eventually lead to death. Most patients become symptomatic in the neonatal period with a rapidly progressive deterioration and a high mortality rate. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the mitochondrial carrier (TC 2.A.29) family.
Contains 3 Solcar repeats.

Database links:

Entrez Gene: 788 Human

Entrez Gene: 57279 Mouse

Entrez Gene: 117035 Rat

Omim: 212138 Human

SwissProt: O43772 Human

SwissProt: Q9Z2Z6 Mouse

SwissProt: P97521 Rat

Unigene: 13845 Human

Unigene: 29666 Mouse

Unigene: 3289 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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