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In August 1908, a white mob attacked Springfield, Illinois’s Black community, destroying homes and businesses and lynching two Black men. National outrage over the violence helped spur the movement that created the NAACP the following year.
MORE →Reflects the personal views, recollections, and perspective of the author, Mike Davis.
This is a personal recollection on the Move fire on May 13, 1985
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| Identifiers | ||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Aliases | ISCU, 2310020H20Rik, HML, ISU2, NIFU, NIFUN, hnifU, iron-sulfur cluster assembly enzyme | |||||||||||||||||||||||||||||||||||||||||||||||||||||
| External IDs | OMIM: 611911; MGI: 1913633; GeneCards: ISCU | |||||||||||||||||||||||||||||||||||||||||||||||||||||
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Iron-sulfur cluster assembly enzyme ISCU, mitochondrial is a protein that in humans is encoded by the ISCU gene.[5] It encodes an iron-sulfur (Fe-S) cluster scaffold protein involved in [2Fe-2S] and [4Fe-4S] cluster synthesis and maturation.[6][7][8][9] A deficiency of ISCU is associated with a mitochondrial myopathy with lifelong exercise intolerance where only minor exertion causes tachycardia, shortness of breath, muscle weakness and myalgia.[10]
ISCU is located on the q arm of chromosome 12 in position 23.3 and has 8 exons.[7] ISCU, the protein encoded by this gene, is a member of the NifU family. It is an iron-sulfur transferase that contains binding sites for [2Fe-2S] and [4Fe-4S] clusters. ISCU contains a transit peptide, 4 beta strands, 4 alpha helixes, and 4 turns.[8][9] Alternative splicing results in transcript variants encoding different protein isoforms that localize either to the cytosol or to the mitochondrion. A pseudogene of this gene is present on chromosome 1.[7]
ISCU encodes a component of the iron-sulfur (Fe-S) cluster scaffold responsible for the synthesis and maturation of [2Fe-2S] and [4Fe-4S] clusters. Fe-S clusters are cofactors that play a role in the function of a diverse set of enzymes, including those that regulate metabolism, iron homeostasis, and oxidative stress response. In one process, the [2Fe-2S] cluster transiently assembles on ISCU and is then transferred to GLRX5 in a cysteine desulfurase complex NFS1-LYRM4/ISD11 dependent process.[7][6][8][9]
ISCU has two isoforms, isoform 1, which is found in the mitochondrion and isoform 2, which is found in the nucleus and cytoplasm.[8][9]
ISCU mutations have been found in patients with a mitochondrial myopathy called hereditary myopathy with lactic acidosis (HML).[11] It is also known as hereditary myopathy with exercise intolerance, Swedish type;[11] myopathy with deficiency of succinate dehydrogenase and aconitase;[11] myoglobinuria due to abnormal glycolysis;[11] myopathy with deficiency of ISCU;[10] Larsson–Linderholm syndrome;[12] and Linderholm myopathy.[13]
This disease is a result of a deficiency of ISCU that corresponds to the deficiency of mitochondrial iron-sulfur proteins and impaired muscle oxidative metabolism.[7] Characteristics of mitochondrial myopathy with deficiency of ISCU may include lifelong exercise intolerance in which exertion can cause tachycardia, dyspnoea, cardiac palpitations, shortness of breath, fatigue, pain of active muscles, rhabdomyolysis, myoglobinuria, elevated lactate and pyruvate, decreased oxygen utilization, may have a pseudoathletic appearance of hypertrophic calf muscles, and possibly weakness.[14][15][8][9][10][16] Primarily affecting the skeletal muscles, rarely there is also hypertrophic cardiomyopathy and ptosis (drooping eyelid).[11][17]
Biopsy of skeletal muscle shows deficiency of succinate dehydrogenase and aconitase, abnormal iron deposition and lipid droplet accumulation in the mitochondria. Histochemical studies show impaired respiratory chain complexes I, II, and III, impairing oxidative phosphorylation. Electromyography normal or myopathic increased polyphasic MUAPs. EMG results may be dynamic: more likely to have increased polyphasic MUAPs after exercise.[14] There has been documented temporary restoration of succinate dehydrogenase in regenerating muscle after an episode of rhabdomyolysis; however, the effect does not last.[18]
The disease is limited to muscle, with fibroblasts from skin biopsy being unaffected. As non-muscle tissues are unaffected, this necessitates the need for muscle biopsy when DNA testing using saliva or blood is inconclusive.[19][17]
This disorder has been associated with several different mutations and is inherited predominantly in an autosomal recessive manner. It was originally believed to affect only those of northern Swedish ancestry, however the disease has been found in those of Norwegian and Finnish decent as well. The carrier rate in northern Sweden has been estimated at 1:188.[15] ISCU deficiency has been linked to pathogenic variants including intronic variants c.418+382G>C, g.7044G>C,[19] and IVS5+382 G>C[18] as well as a c.149G>A missense mutation in exon 3.[20] The intronic mutations have been suggested to activate a cryptic splice site, resulting in the production of a splice variant that encodes a putatively non-functional protein.[10]
In 2016, an Italian male was found to have a de novo autosomal dominant mutation, c.287G>T (p.Gly96Val) in the ISCU gene, that caused hereditary mitochondrial myopathy with lactic acidosis (HML). This mutation resulted in a similar phenotype as seen in the recessive form of the disease.[17]
ISCU has been shown to have 235 binary protein-protein interactions including 79 co-complex interactions. ISCU appears to interact with ISCS, NUP62, SDHB, HPRT1, CCDC172, GOLGA2, IKZF1, KRT40, AGTRAP, NECAB2, FAM9B, BANP, LNX1, MID2, GOLGA6L9, ccdc136, KRT34, SPERT, PICK1, YWHAB, SFN, mbl, E7, dnaX, hscB, MAPk-Ak2, hale, and cv-c.[21]
This article incorporates text from this source, which is in the public domain.
This article incorporates text available under the CC BY 4.0 license.
This article incorporates text from this source, which is in the public domain.
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This article incorporates text from the United States National Library of Medicine, which is in the public domain.
Source: Wikipedia. Article content is retrieved live through the MediaWiki API.
Iron-sulfur cluster assembly enzyme ISCU, mitochondrial is a protein that in humans is encoded by the ISCU gene. It encodes an iron-sulfur (Fe-S) cluster scaffold protein involved in [2Fe-2S] and [4Fe-4S] cluster synthesis and maturation. A deficiency of ISCU is associated with a mitochondrial myopathy with lifelong exercise intolerance where only minor exertion causes tachycardia, shortness of breath, muscle weakness and myalgia.
Bassariscus is a genus in the family Procyonidae. There are two extant species in the genus: the ringtail or ring-tailed cat (B. astutus) and the cacomistle (B. sumichrasti). Genetic studies have indicated that the closest relatives of Bassariscus are raccoons, from which they diverged about 10 million years ago in the Tortonian Age of the Miocene. The two lineages of Bassariscus are thought to have separated after only another two million years, making it the extant procyonid genus with the earliest diversification. Later diversification in the genus in the Pliocene and Pleistocene saw the emergence of two extinct species, Bassariscus casei and Bassariscus sonoitensis, respectively. Due to the more digitigrade stance of their legs compared to the plantigrade stance of other members of Procyonidae, some taxonomies place the genus as a separate family, Bassaricidae.. The name is a Greek word for fox ("bassaris") with a Latinized diminutive ending ("-iscus"). The genus was named by Elliott Coues in 1887, having previously been described by Lichtenstein in 1830 under the name Bassaris. Coues proposed the word "bassarisk" as the English term for animals in this genus. Its habitat includes semi-arid areas in the southwestern United States, the whole of Mexico, as well as moist tropical forests in Central America.
Cysteine desulfurase, mitochondrial is an enzyme that in humans is encoded by the NFS1 gene. Iron-sulfur clusters are required for the function of many cellular enzymes. The protein encoded by this gene supplies inorganic sulfur to these clusters by removing the sulfur from cysteine, creating alanine and a protein-bound hydropersulfide in the process. The terminal sulfur atom of the hydropersulfide is transferred to the iron-sulfur cluster scaffold protein ISCU, mediated by the accessory protein frataxin. This gene uses alternate in-frame translation initiation sites to generate mitochondrial forms and cytoplasmic/nuclear forms. Selection of the alternative initiation sites is determined by the cytosolic pH. The encoded protein belongs to the class-V family of pyridoxal phosphate-dependent aminotransferases.
Isku (Quechua for lime, Hispanicized spelling Iscu) is a mountain in the Andes of Peru, about 5,000 metres (16,404 ft) high. It is located in the Lima Region, Oyón Province, Oyón District. Isku lies southeast of Yana Uqhu.
Before the 1921 destruction of Tulsa’s Greenwood District, Black residents had created a remarkable center of business and community life. The district included stores, professional offices, entertainment venues and homes owned by Black citizens. Understanding Greenwood means learning what was built—not only what was burned.
MORE →Madam C.J. Walker
